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免疫性血小板减少症 (ITP) - 血液紊乱 - 《默沙东诊疗手册大众版》

免疫性血小板减少症 (ITP) - 血液紊乱 - 《默沙东诊疗手册大众版》

默沙东 诊疗手册

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血小板疾病

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免疫性血小板减少症 (ITP)

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血小板疾病概述

血小板减少症概述

溶血性尿毒症综合征 (HUS)

血栓性血小板减少性紫癜 (TTP)

免疫性血小板减少症 (ITP)

血小板功能障碍

血管性血友病

免疫性血小板减少症 (ITP)

（特发性血小板减少性紫癜；免疫性血小板减少症）

作者：

David J. Kuter

, MD, DPhil, Harvard Medical School

全面审查/修订6月 2022

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瘀斑（瘀伤）

瘀点（皮肤）

瘀点（口）

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免疫性血小板减少症 (ITP) 是一种由血小板（凝血细胞）数目减少引起的出血性疾病，并且患者不存在其他可影响血小板的疾病。在 ITP 中，免疫系统会针对患者自身的血小板产生抗体并破坏它们。 患者的皮肤上可能会有微小的紫色斑点（瘀点），并且容易流血。 诊断方法是通过血液检查检测血小板计数。 可给予皮质类固醇或其他药物来阻止对血小板的破坏。 使用可增加血小板生成量的药物对某些患者有帮助。 在成年患者中，医生有时会切除脾脏。 （也可参见 血小板疾病概述 血小板疾病概述 血小板（有时称为凝血细胞）是在骨髓中生成、在血流中循环的细胞碎片，可帮助 凝血。促血小板生成素主要在肝脏生成，可刺激骨髓合成大细胞（巨核细胞），继而利用其细胞体（细胞质）内的原料生成血小板。未参与凝血的血小板会循环 7 到 10 天，随后会被破坏。约有三分之一的血小板始终储存在脾内。... 阅读更多 和 血小板减少症概述 血小板减少症概述 血小板减少症是指血液内的血小板（凝血细胞）计数低，这会增加出血风险。 如果骨髓合成的血小板过少或者血小板过多被破坏或积聚在肿大的脾脏内，会发生血小板减少症。 可见皮内出血和瘀青。 医生利用血液检查作出诊断并确定病因。 有时需要治疗（例如血小板输注，使用泼尼松和增加血小板生成量的药物，或者切除脾脏）。 阅读更多 。） 血小板是骨髓合成的细胞，在血流中循环，有助于 凝血 如何凝血 止血是指机体使受损血管停止出血的过程。止血过程包括凝血。 凝血不足可能会导致轻微损伤过多出血 过度凝血可能会阻塞没有出血的血管 因此，身体具有控制机制，以限制凝血和溶解不再需要的血栓。这个系统任何一部分的异常都会导致 过度出血或 过度凝血，两者都很危险。如果凝血功能差，即使血管的轻微损伤都可能会导致... 阅读更多 。通常，血液含有约 140,000 至 440,000 个血小板／微升（140 至 440 × 109／升）。如果每微升血液内的血小板计数降至约 50,000（低于 50 × 10 9／升）个血小板以下，甚至较为轻微的损伤也会引起出血。然而，在每微升血液的血小板计数降至 10,000 至 20,000（10 至 20 × 109／升）个血小板以下之前，一般不会出现最严重的出血风险。在如此低的血小板水平下，即使没有任何可辨识的损伤也可能会出血。 免疫性血小板减少症是 抗体 抗体 机体的其中一道防线（ 免疫系统）涉及穿过血流并进入组织、寻找并攻击微生物及其它入侵者的 白细胞。（另见 免疫系统概述。） 这层防御具有两个组成部分： 先天免疫 获得性免疫 获得性（适应性或特异性）不是与生俱来的，而是后天获得的。它是学得的。当一个人的免疫系统遇到外来入侵者并识别出非自身物质（抗原）时，学习过程便开始了。然后，获得性免疫的成分将学习攻击每种抗原的最佳方法，并开始对该抗原产生记忆。获得性免疫也被称为特异性免疫，这是因为它仅限... 阅读更多 形成并破坏身体血小板的疾病。形成抗体的原因尚不清楚；但在儿童中，ITP 经常发生在病毒感染之后。尽管骨髓可能会增加血小板生成以代偿其受到的破坏，但血小板通常供不应求。有时，破坏血小板的抗体也会攻击骨髓，导致血小板生成量下降。 在成人中，ITP 通常长久存在（慢性疾病）。在儿童中，ITP 往往会自然消除。 ITP 的症状 有些人可能没有免疫性血小板减少症的症状。而另一些人则可能突然或逐渐出现出血症状。疲劳在慢性 ITP 患者中常见。 皮内出血可能是血小板计数低最先表现出的征象。小腿皮肤内经常出现许多小红点（瘀点），并且即使轻微损伤也可能导致青一块紫一块的瘀伤（瘀斑或紫癜）。还可能出现牙龈出血、便血或尿血。月经出血或鼻出血可能异常量大。出血难以止住。 皮内出血 瘀斑（瘀伤） 这里的瘀斑是腿上看到的紫色大块瘀伤。 P. MARAZZI 博士／科学图片库 瘀点（皮肤） 瘀点是皮肤上的红色小斑点。 经出版商允许。摘自 Deitcher S. 临床血液病学图谱。由 JO Armitage 编辑。费城，《当代医学》，2004 年。 瘀点（口） 瘀点是口腔中的红色小斑点。 P. MARAZZI 博士／科学图片库 随着血小板数目的减少，出血加重。血小板极少的患者可能会出现消化道大量失血，或在罕见情况下，即使未受伤也可能发生危及生命的 脑内出血 症状 。脑内出血时，可出现头痛和其他神经系统症状。 ITP 的诊断 进行血液检查以检测血小板计数和凝血功能 进行检查以排除可能引起血小板计数低和出血的其他疾病 如果每微升血液的血小板计数低于 100,000（低于 100 × 109/升）并且红细胞或白细胞数目没有发生类似下降，此外也不存在可明确解释血小板减少症的其他病因（如感染或使用某些药物），那么医生会诊断为免疫性血小板减少症（ITP；见表）。尚无公认的检查项目可确认某人患有 ITP。 血小板计数可使用自动计数仪测量以确定血小板减少症的严重程度，并且必须采集一份血样在显微镜下检查，以便为确定病因提供线索。医生需要在显微镜下检查血样，以将 ITP 与 血栓性血小板减少性紫癜 血栓性血小板减少性紫癜 (TTP) 血栓性血小板减少性紫癜 (TTP) 是一种严重疾病，全身形成小血栓，阻塞血液流向脑、心、肾等重要器官。 具体症状与体内形成血栓的部位有关。 诊断依据是患者的症状和血液检查结果。 TTP 的治疗方法是进行血浆置换，使用皮质类固醇、利妥昔单抗，极少数情况下使用卡普赛珠单抗。 （也可参见 血小板疾病概述和 血小板减少症概述。） 阅读更多 (TTP) 和 溶血性尿毒症综合征 溶血性尿毒症综合征 (HUS) 溶血性尿毒症综合征 (HUS) 是一种常见于儿童的严重疾病，全身形成小血栓，阻塞血液流向脑、心脏、肾脏等重要器官。 具体症状与体内形成血栓的部位有关。 诊断依据是患者的症状和血液检查结果。 HUS 的治疗可支持重要的身体功能，有时需要血液透析，艾库组单抗对有些患者有帮助。 （也可参见 血小板疾病概述和 血小板减少症概述。） 阅读更多 (HUS) 鉴别开来。TTP 和 HUS 也是可通过破坏血小板引起血小板减少症的疾病。 罕见情况下，需要从骨髓取样并在显微镜下检查（骨髓活检和穿刺 骨髓检查 红细胞、大多数 白细胞和 血小板在骨髓（骨腔内的软脂肪组织）中产生。有时必须检查骨髓样本，明确血细胞异常或者某一类血细胞太少或太多的原因。医生采集的骨髓样本有两种不同类型： 骨髓穿刺：将针头插入骨髓，吸出体液和细胞 骨髓粗针穿刺活检：使用芯针装置（类似于大孔径针头），取出一块完整骨髓 骨髓穿刺液可显示骨髓中有哪些细胞、是正常还是异常，并提供有关细胞大小、体积和其他特征的信息。医生可对样本做一些特殊检测，例如细菌、真菌或病毒培养，染色体分... 阅读更多 ），以了解血小板生成的情况。 ITP 的治疗 皮质类固醇 静脉注射免疫球蛋白、促血小板生成素受体激动剂或免疫抑制剂（例如，利妥昔单抗、硫唑嘌呤或麦考酚酯） 有时需切除脾脏 罕见情况下需输注血小板 在 ITP 中，抗体对血小板的破坏效应可以通过应用皮质类固醇（例如泼尼松）或静脉输注免疫球蛋白来暂时阻断，从而使血小板数目增加。经过该治疗后，儿童通常会在几周到几个月内恢复。 约三分之一的成年患者会在治疗后的第一年内恢复，但大部分患者则不会。对于皮质类固醇疗效不足或依赖于皮质类固醇的成年患者可能需要给予其他药物，以增加血小板生成（促血小板生成素受体激动剂）或抑制免疫系统（包括利妥昔单抗、硫唑嘌呤、环磷酰胺、环孢菌素或麦考酚酯）。福他替尼是另一种备选药物，可在其他药物无效时使用。 促血小板生成素受体激动剂（例如罗米司亭、艾曲波帕和阿伐曲泊帕）可加快血小板生成速度，有效性可持续多年。这些药物对于不能接受（或不愿意接受）脾切除术的患者特别有益。 有些 ITP 成年患者（但通常不适用于儿童患者）会受益于手术切除脾脏（脾切除术）。脾脏会清除血流中的异常血小板，因此，有时切脾可增加血小板数目。脾切除术的缺点包括形成血栓、发生癌症以及出现某些危及生命的感染（例如 肺炎球菌感染 肺炎球菌感染 肺炎球菌感染是由 革兰氏阳性球形菌（球菌）（参见图 细菌如何塑形）肺炎链球菌（肺炎球菌）引起的感染。这些细菌通常引起肺炎、脑膜炎、鼻窦炎和中耳感染。 感染者咳嗽或打喷嚏时可将肺炎球菌散布在空气中。 肺炎球菌感染通常引起发热和身体不适的感觉，其它症状取决于感染的身体部位。 诊断依据包括症状或对感染物样本的细菌鉴定。 青霉素或其他抗生素治疗通常有效。 阅读更多 ）的风险增加。患者在脾切除术后可能需要使用某些抗生素或疫苗，以减小（但不会彻底消除）感染风险。与脾切除术相比，药物治疗的使用比例呈增长态势。 如果患者发生了危及生命的出血，则可能会在静脉输注皮质类固醇和/或免疫球蛋白的基础上输注血小板。

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Merck & Co., Inc., Rahway, NJ, USA 及其附属公司。保留所有权利。（美加以外地区称为默沙东）是努力改善全球福祉的健康护理引领者。从开发治疗和预防疾病的新疗法，到满足人们需求，我们致力于改善全世界人们的健康和福祉。 本手册于 1899 年作为一项社区服务首次出版。 在北美之外，这一重要遗留资源仍以 MSD 手册的形式继续提供。详细了解我们对全球医学知识的承诺。

 

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成人ITP指南（2020年版） - 知乎

成人ITP指南（2020年版） - 知乎切换模式写文章登录/注册成人ITP指南（2020年版）阮大大第一部分成人原发免疫性血小板减少症01概述原发免疫性血小板减少症（primary immune thrombocytopenia, ITP）是一种获得性自身免疫性出 血性疾病，以无明确诱因的孤立性外周血血小板计数减少为主要特点。目前国内尚无基于人口基数的ITP流行病学数据，国外报道的成人ITP年发病率为（2～10）/10 万［1- 2］，60 岁以上老年人是高发群体，育龄期女性略高于同年龄组男性。该病临床表现变化较大，无症状血小板减少、皮肤黏膜出血、严重内脏出血、致命性颅内出血均可发生。老年患者致命性出血发生风险明显高于年轻患者。部分患者有乏力、焦虑表现。ITP主要发病机制是血小板自身抗原免疫耐受性丢失，导致体液和细胞免疫异常活化，共同介导血小板破坏加速及巨核细胞产生血小板不足［3］ 。中华医学会血液学分会血栓与止血学组分别于2009、2011、2012、2016 年对“成人ITP诊治的中国专家共识”进行了4次更新［4-7］，2018年发布了中国成人 ITP 治疗指南［8］。ITP国际工作组及美国血液学会（ASH）在2019年均对其既往发布的共识、指南进行了相应更新［9-10］。结合国内临床研究进展及实际情况，中华医学会血液学分会血栓与止血学组召集国内专家对中国成人ITP诊治指南（2018版）进行修订，旨在为成人ITP诊治提供最新的临床指导。02诊断要点ITP的诊断仍基于临床排除法，须除外其他原因所致血小板减少。除详细询问病史及细致体检外，其余诊断要点包括： 1. 至少连续2次血常规检查示血小板计数减少，外周血涂片镜检血细胞形态无明显异常。 2. 脾脏一般不增大。3. 骨髓检查：ITP 患者骨髓细胞形态学特点为巨核细胞增多或正常，伴成熟障碍［11］。 4. 须排除其他继发性血小板减少症：自身免疫性疾病、甲状腺疾病、淋巴系统增殖性疾病、骨髓增生异常综合征（MDS）、再生障碍性贫血（AA）、各种恶性血液病、肿瘤浸润、慢性肝病、脾功能亢进、普通变异型免疫缺陷病（CVID）、感染、疫苗接种等所致继发性血小板减少；血小板消耗性减少； 药物所致血小板减\少；同种免疫性血小板减少；妊娠期血小板减少；先天性血小板减少及假性血小板减少［12］。5. 诊断ITP的特殊实验室检查：（1）血小板糖蛋白特异性自身抗体：对抗体介导的免疫性血小板减少症有较高的特异性，可鉴别免疫性与非免疫性血小板减少，但不能区分原发与继发免疫性血小板减少［13-14］。（2）血清血小板生成素（TPO）水平测定：有助于ITP（TPO水平正常）和骨髓衰竭性疾病（TPO水平升高）的鉴别诊断［15］。对疑诊ITP患者推荐的基本评估和特殊实验室检查详见表1。6. 出血程度分级：应用出血评分系统量化ITP患者出血情况及风险评估。该系统分为年龄和出血症状两个部分（表2）。ITP患者的出血评分＝年龄评分+出血症状评分（所有出血症状中最高的分值）03疾病的分期、分级依据病程长短，ITP分为以下三期［16］ （1）新诊断的ITP：确诊后3个月以内的患者；（2）持续性 ITP：确诊后3～12个月血小板持续减少的患者，包括未自发缓解和停止治疗后不能维持完全缓解的患者；（3）慢性ITP：血小板持续减少超过12个月的患者。 重症 ITP：血小板计数＜10×109/L伴活动性出血，或出血评分≥5分。 难治性ITP：指对一线治疗药物、二线治疗中的促血小板生成药物及利妥昔单抗治疗均无效，或脾切除无效/术后复发，进行诊断再评估仍确诊为ITP的患者。04治疗原则及方案根据美国国立临床诊疗指南数据库证据分级系统对证据进行分级及推荐。证据等级定义如下：Ⅰa级：源于随机对照试验结果的Meta分析；Ⅰb级：源于≥1个的随机对照试验；Ⅱa级：源于≥1个的设计良好的对照研究；Ⅱb 级：源于≥1个的设计良好的类试验研究；Ⅲ级：源于设计良好的非试验描述性研究（如对比性研究、相关研究）；Ⅳ级：源于专家委员会报告或权威专家经验。推荐等级标准定义如下：A级：源于Ⅰa、Ⅰb级证据，要求推荐方案论述总体质量好、一致性强，且内容中包含≥1个的随机对照研究；B级：源于Ⅱa、Ⅱb、Ⅲ级证据，要求推荐方案进行了较好的非随机化临床研究；C级：源于Ⅳ级证据，推荐内容证据源于专家委员会报告或权威专家临床经验或意见，缺乏临床研究直接证据。01治疗原则ITP的治疗遵循个体化原则，鼓励患者参与治疗决策，兼顾患者意愿，在治疗不良反应最小化基础上提升血小板计数至安全水平，减少出血事件，关注患者健康相关生活质量（HRQoL）。1. 对于血小板计数≥30×109 /L、无出血表现且不从事增加出血风险工作、无出血风险因素的ITP患者，可予以观察随访。若患者有活动性出血症状（出血症状评分≥2分），不论血小板减少程度如何，都应给予治疗。 2. 增加出血风险因素：①高龄和长ITP病史；②血小板功能缺陷；③凝血障碍；④高血压；⑤外伤或手术；⑥感染；⑦抗血小板、抗凝或非甾体类药物治疗。 3. ITP患者部分临床常规操作或手术以及接受药物治疗时血小板计数参考值［10］：龈上洁治术及深度清洁：PLT≥（20～30）×109/L；拔牙或补牙：PLT≥（30～50）×109/L；小手术：PLT≥50×109/L；大手术： PLT≥80×109/L；神经外科大手术：PLT≥100×109 /L；单一抗血小板或抗凝治疗：PLT≥（30～50）×109 /L；抗血小板联合抗凝治疗：PLT≥（50～70）×109 /L。02紧急治疗ITP患者发生危及生命的出血（如颅内出血）或需要急症手术时，应迅速提升血小板计数至安全水平。可给予静脉注射免疫球蛋白（IVIg）1 g·kg-1·d-1×1～2 d（C 级 推 荐）、静脉甲泼尼龙1000mg/d×3d和重组人血小板生成素（rhTPO）300U·kg-1·d-1 皮下注射治疗。上述措施可单用或 联合应用，并及时予以血小板输注（Ⅲ/Ⅳ级证据）。 其他紧急治疗措施包括长春碱类药物、急症脾 切除、抗纤溶药物、控制高血压、口服避孕药控制月 经过多、停用抗血小板药物等（C级推荐）。03一线治疗1. 糖皮质激素：①大剂量地塞米松（HD-DXM） 40mg/d×4d，口服或静脉给药，无效或复发患者可重复1个周期。治疗过程中注意监测血压、血糖水平，注意预防感染及消化道溃疡。②泼尼松 1mg·kg-1·d-1（最大剂量 80 mg/d，分次或顿服），起效后应尽快减量，6～8周内停用，减停后不能维持疗效患者考虑二线治疗。如需维持治疗，泼尼松的安全剂量不宜超过5 mg/d。2周内泼尼松治疗无效患者应尽快减停。糖皮质激素依赖：指需要5mg/d以上泼尼松或频繁间断应用糖皮质激素维持PLT≥30×109/L和（/或）避免出血。 HD-DXM 治疗7d内反应率明显高于泼尼松，但持续反应率、严重出血改善无明显差异（Ⅰb级证据）［17-18］。高龄、糖尿病、高血压、青光眼等患者应慎用。应用HD-DXM的同时建议给予抗病毒药物，预 防疱疹病毒、乙型肝炎病毒（HBV）等再激活（C级推 荐，Ⅳ级证据）。长期应用糖皮质激素可发生高血 压、高血糖、急性胃黏膜病变等不良反应，部分患者 可出现骨质疏松、股骨头坏死。 注意糖皮质激素对精神健康的影响，定期评估患者治疗期间 HRQoL（抑郁、疲劳、精神状态等）。 HBV-DNA 复制水平较高的患者慎用糖皮质激素，治疗方案的制订可参照2019版《中国慢性乙型肝炎防治指南》［19］。2. IVIg：IVIg主要用于：①紧急治疗；②糖皮质 激素不耐受或有禁忌证的患者；③妊娠或分娩前。推荐400 mg·kg-1·d-1×5d或1g·kg-1 ·d-1×1～2d。有 条件者可行血小板糖蛋白特异性自身抗体检测，有助于IVIg的疗效预判［20］。IgA缺乏和肾功能不全患 者应慎用。04二线治疗1. 促血小板生成药物：包括rhTPO、艾曲泊帕等。此类药物于1～2周起效，有效率可达60％以上，停药后多不能维持疗效，需进行个体化维持治疗。①rhTPO：300U·kg-1·d-1×14d，皮下注射给药，有效患者行个体化维持。治疗14d仍未起效的患者应停药（A级推荐，Ⅰb级证据）［6］。②艾曲泊帕：25mg/d空腹 顿服，治疗2周无效者加量至50mg/d（最大剂量75 mg/d），进行个体化药物调整，维持血小板计数≥50×109/L。最大剂量应用2～4周无效者停药（A 级推荐，Ⅰa 级证据）［21］。对于1种促血小板生成药物无效或不耐受患者，更换其他促血小板生成药物或采用序贯疗法可能使患者获益（Ⅲ级证据）。2. 利妥昔单抗：有效率50％左右，长期反应率为20％～25％（Ⅱa类证据，B级推荐）［22］。有2种常用给药方案：①标准剂量方案：375mg/m2 静脉滴注，每周1次，共4次，通常在首次用药后4～8周内起效。②小剂量方案：100mg静脉滴注，每周1次，共4次，或375mg/m2 静脉滴注1次，起效时间略长。利妥昔单抗原则上禁用于活动性乙型肝炎患者。 3. rhTPO联合利妥昔单抗：推荐rhTPO300U·kg-1·d-1 ×14d；利妥昔单抗100mg静脉滴注，每周1次，共4次。对糖皮质激素无效或复发患者总有效率为79.2％，中位起效时间为7d，6个月持续反应率为67.2％［23］ （A级推荐，Ⅰb级证据）。 4. 注册临床试验（Ⅲ期）5. 脾切除术：适用于糖皮质激素正规治疗无效、泼尼松安全剂量不能维持疗效及存在糖皮质激素应用禁忌证的患者。脾切除应在ITP确诊12～ 24个月后进行（C级推荐），术中留意有无副脾，如发现则应一并切除（C级推荐）［24-25］。术前须对ITP的诊断进行重新评估，建议行单克隆抗体俘获血小板抗原技术（MAIPA）和TPO水平检测。推荐对术后血小板计数上升过高、过快者进行血栓风险评估，对中高危患者给予血栓预防治疗（C级推荐）。有条件的患者脾切除2周前可行疫苗接种（肺炎双球菌、脑膜炎奈瑟菌、流感嗜血杆菌）。05三线治疗目前，有设计良好的前瞻性多中心临床试验支持 的三线治疗方案包括：①全反式维甲酸（ATRA）联合达那唑：ATRA 20mg/d（分2次口服），达那唑 400mg/d（分2次口服），二者联合应用16周。糖皮质激素无效或复发患者的1年持续有效率约为62％，中位起效时间为5周，患者耐受性良好［26］（B级推荐，Ⅰb级证据）。②地西他滨：3.5mg·m-2·d-1 ×3d静脉滴注，间隔3周后再次给药，共3～6个周期，治疗3个周期无效患者应停用。总有效率约为50％，6个月持续反应率约为40％，不良反应轻微［27］(B级推荐，Ⅲ级证据）。06其他药物硫唑嘌呤、环孢素A、达那唑、长春碱类等药物缺乏足够的循证医学证据，可根据医师经验及患者状况进行个体化选择。成人ITP诊治流程见下图。05疗效判断1. 完全反应（CR）：治疗后血小板计数≥100× 109/L且无出血表现。 2. 有效（R）：治疗后血小板计数≥30×109/L，比基础血小板计数增加至少2倍，且无出血表现。 3. 无效（NR）：治疗后血小板计数＜30×109 /L，或血小板计数增加不到基础值的2倍，或有出血。 4. 复发：治疗有效后，血小板计数降至30×109 /L以下，或降至不到基础值的2倍，或出现出血症状。 5. 持续有效：患者疗效维持至开始治疗后6个月及以上。 6. 早期反应：治疗开始1周达到有效标准。 7. 初步反应：治疗开始1个月达有效标准。8. 缓解：治疗开始后12个月时血小板计数≥ 100×109/L。 在定义CR或R时，应至少检测2次血小板计数，间隔至少7d。定义复发时至少检测2次，其间至少间隔1d。第二部分妊娠合并原发免疫性血小板减少症01概述妊娠合并ITP的年发病率约为8/10万，病情复杂多变，疾病通常随妊娠进展而加重。回顾性研究显示多数妊娠合并ITP患者没有出血症状，而有出血症状的孕妇中，90％表现为轻中度皮肤或黏膜出血［28］。妊娠合并ITP诊断步骤与非妊娠患者类似，同时须鉴别妊娠相关血小板减少症（妊娠期血小板减少症、子痫前期等）。当血小板减少孕妇有可疑ITP病史或血小板计数＜80×109/L时，应进一步排查妊娠合并ITP的可能（C级推荐）。02诊断及鉴别诊断妊娠合并ITP同样是排除性诊断。除详细的病 史采集和体格检查外，应进行的实验室检查包括：外周血全血细胞及网织红细胞计数、凝血检查、肝肾功能、甲状腺功能、抗核抗体谱、抗磷脂抗体、HBV/HCV/HIV抗体、IgA/IgG/IgM水平和外周血涂片镜检（C级推荐）。如不伴有血细胞形态异常，原则上不推荐骨髓检查（C级推荐）。诊断妊娠合并ITP时，需要鉴别的疾病包括：妊娠期血小板减少症、子痫前期、HELLP综合征、继发免疫性血小板减少症、感染相关血小板减少症（HCV、HIV、巨细胞病毒）、药物相关血小板减少症、弥散性血管内凝血（DIC）、血栓性血小板减少性紫癜/溶血性尿毒综合征（TTP/HUS）、骨髓增生异常综合征、再生障碍性贫血、营养缺乏症等［10］。03治疗原则及方案妊娠合并ITP的治疗目的是降低妊娠期出血及 与血小板减少相关的区域麻醉和分娩出血并发症风险。除分娩期外，妊娠合并ITP的治疗指征与非妊娠患者一致。当患者血小板计数＜30×109/L且伴 活动性出血或准备分娩时，应提升血小板计数至相对安全水平。自然分娩和剖宫产的血小板安全水平：自然分娩≥50×109/L，剖宫产≥80×109/L。01一线治疗1. 口服糖皮质激素：泼尼松 20mg/d，起效3周后逐渐减量，以5～10mg/d剂量维持，有效率不足40％［29］。用药过程中注意监测患者血压、血糖、血脂、精神状态等。分娩后严密监测产妇血小板水 平，并缓慢减少糖皮质激素用量，以免对产妇精神状态造成不利影响（Ⅳ级推荐）。 2. IVIg：适用于糖皮质激素效果不佳、有严重不良反应或需紧急提高血小板水平的患者（B级推荐）［29］。推荐1g/kg单次给药或400mg·kg-1·d-1×3～5d。起效时间优于糖皮质激素，不能维持长期疗效。02二线治疗对于初始治疗失败的妊娠合并ITP患者，可采取的进一步治疗措施包括：①糖皮质激素联合IVIg：对泼尼松或IVIg单药治疗无效及泼尼松维持治疗中失去反应的患者，二者联合可能有效（C级推荐）。或给予大剂量甲泼尼龙+IVIg治疗（C级推荐）。②rhTPO：对初始治疗无效的晚期妊娠合并ITP患者，可考虑给予rhTPO［30］（B级推荐，Ⅲ级证据）。（执笔：刘新光、侯明）参与指南制定和讨论的专家（同一单位多个专家按照姓氏首字母排序）：中国科学技术大学附属第一医院安徽省立医院（郑昌成）；安徽医科大学第一附属医院（曾庆曙）；北京大学人民医院（付海霞、张晓辉）；首都医科大学附属北京儿童医院（吴润晖）；中国医学科学院北京协和医院（赵永强、朱铁楠）；成都市妇女儿童中心医院（周敏）；大连医科大学附属第一医院（申静枝）；陆军军医大学新桥医院（孔佩艳）；西北大学附属医院/西安市第三医院（舒汨汨）；福建医科大学附属协和医院（陈英玉）；广西医科大学第一附属医院（程鹏）；广州医科大学附属第二医院（冯莹）；广州医科大学附属第二医院（叶絮）；哈尔滨医科大学附属第四医院（洪珞珈）；哈尔滨医科大学附属第一医院（苏雁华）；海南省人民医院（吴从明）；河南省肿瘤医院（周虎）；华北理工大学附属医院（闫振宇）；华中科技大学同济医学院附属协和医院（胡豫、梅恒、唐亮）；江苏省苏北人民医院（孙梅）；解放军总医院（黄文荣）；昆明医科大学第二附属医院（周泽平）；南昌大学第一附属医院（黄瑞滨）；南方医科大学南方医院（孙竞、叶洁瑜）；南京鼓楼医院（周荣富）；青海省人民医院（冯建明）；山东大学齐鲁医院（侯宇、侯明、刘新光、彭军、石艳、徐从高）；山西医科大学第二医院（杨林花、张睿娟）；上海复旦大学附属中山医院（程 韵枫）；上海交通大学医学院附属瑞金医院（武文漫）；四川大学华西医院（龚玉萍）；四川省人民医院（王春森）；苏州大学附属第一医院（戴克胜、韩悦、季顺东、余自强）；新疆医科大学第一附属医院（郭新红）；徐州医科大学附属医院（乔建林）；云南省第一人民医院（杨同华）；浙江省中医院（沈建平、周郁鸿）；中国医学科学院血液病医院（刘晓帆、薛峰、杨仁池、张磊）；中南大学湘雅医院（陈方平、彭捷）；中南大学湘雅二医院（张广森、邓明扬）；中山大学孙逸仙纪念医院（马丽萍）；ITP家园（高航、孟寒冰）参 考 文 献 ［1］ Moulis G, Palmaro A, Montastruc JL, et al. 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Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group［J］. Blood, 2009, 113（11）: 2386-2393. DOI: 10.1182/blood-2008-07-162503. ［17］ Wei Y, Ji XB, Wang YW, et al. High- dose dexamethasone vs prednisone for treatment of adult immune thrombocytopenia: a prospective multicenter randomized trial［J］. Blood, 2016, 127 （3）: 296-302. DOI: 10.1182/blood-2015-07-659656. ［18］ Godeau B. High- dose dexamethasone or oral prednisone for immune thrombocytopenia?［J］. Lancet Haematol, 2016, 3（10）: e453-e454. DOI: 10.1016/S2352-3026（16）30124-7. ［19］ 中华医学会感染病学分会, 中华医学会肝病学分会. 慢性乙型 肝炎防治指南（2019 年版）［J］. 中华传染病杂志, 2019, 37 （12）: 711-736. DOI: 10.3760/cma.j.issn.1000-6680.2019. 12.003. ［20］Peng J, Ma SH, Liu J, et al. Association of autoantibody specific-ity and response to intravenous immunoglobulin G therapy in immune thrombocytopenia: a multicenter cohort study［J］. 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Acta Haematol, 2016, 135（3）: 162- 171. DOI: 10.1159/000442703. ［25］ Tada K, Ohta M, Saga K, et al. Long-term outcomes of laparoscopic versus open splenectomy for immune thrombocytopeni［J］. Surg Today, 2018, 48（2）: 180-185. DOI: 10.1007/s00595- 017-1570-2. ［26］ Feng FE, Feng R, Wang M, et al. Oral all-trans retinoic acid plus danazol versus danazol as second- line treatment in adults with primary immune thrombocytopenia: a multicentre, randomised, open- label, phase 2 trial［J］. Lancet Haematol, 2017, 4（10）: e487-e496. DOI: 10.1016/S2352-3026（17）30170-9. ［27］ Zhou H, Qin P, Liu Q, et al. A prospective, multicenter study of low dose decitabine in adult patients with refractory immune thrombocytopenia［J］. Am J Hematol, 2019, 94（12）: 1374- 1381. DOI: 10.1002/ajh.25646. ［28］ ACOG Practice Bulletin No. 207 Summary: Thrombocytopenia in Pregnancy［J］. Obstet Gynecol, 2019, 133（3）: 589-591. DOI: 10.1097/AOG.0000000000003101. ［29］ Sun D, Shehata N, Ye XY, et al. Corticosteroids compared with intravenous immunoglobulin for the treatment of immune thrombocytopenia in pregnancy［J］. Blood, 2016, 128（10）: 1329- 1335. DOI: 10.1182/blood-2016-04-710285. ［30］Kong ZY, Qin P, Xiao S, et al. A novel recombinant human thrombopoietin therapy for the management of immune thrombocytopenia in pregnancy［J］. Blood, 2017, 130（9）: 1097-1103. DOI: 10.1182/blood-2017-01-761262.发布于 2022-06-07 19:10血小板减少性紫癜​赞同 12​​添加评论​分享​喜欢​收藏​申请

原发性血小板减少性紫癜_百度百科

小板减少性紫癜_百度百科 网页新闻贴吧知道网盘图片视频地图文库资讯采购百科百度首页登录注册进入词条全站搜索帮助首页秒懂百科特色百科知识专题加入百科百科团队权威合作下载百科APP个人中心收藏查看我的收藏0有用+10原发性血小板减少性紫癜播报上传视频免疫性综合病征原发性血小板减少性紫癜（Idiopathic thrombocytopenic purpura,ITP)是一种免疫性综合病征，是常见的出血性疾病。特点是血循环中存在抗血小板抗体，使血小板破坏过多，引起紫癜；而骨髓中巨核细胞正常或增多，幼稚化。临床上可分为急性及慢性两种，二者发病机理及表现有显著不同。中医病名原发性血小板减少性紫癜外文名Idiopathic thrombocytopenic purpura,ITP多发群体急性多为10岁以下儿童；慢性多为多为20～50岁女性常见症状皮肤，黏膜自发性出血，血小板减少，骨髓巨核细胞数正常或增多，出血时间延长，血块收缩不良，束臂试验阳性目录1概述▪定义▪英文名称▪其他名称2临床表现▪急性型▪慢性型3治疗▪一般治疗▪西医治疗▪中医治疗▪饮食疗法▪猪皮茅根蜜饮猪▪藕节二叶汤▪猪蹄爪甲地黄汤4病因及发病机制5实验室检查▪血象▪骨髓象▪免疫学检查▪其他6诊断及鉴别诊断▪国内诊断标准▪诊断要点7原发性血小板减少性紫癜的危害8病程及预后9病因病理10辩证分型▪血热妄行症状▪阴虚火旺症状▪气不摄血症状11分型治疗12注意事项13护理预防概述播报编辑原发性血小板减少性紫癜又称为特发性血小板减少性紫癜，即自身免疫性血小板减少性紫癜，是最常见的出血性疾病之一。其主要临床特点是：皮肤，黏膜自发性出血，血小板减少，骨髓巨核细胞数正常或增多，出血时间延长，血块收缩不良，束臂试验阳性。本病的特征是血小板寿命缩短，骨髓中巨核细胞增多伴成熟障碍，脾脏无明显肿大。本病分为急性型与慢性型两种类型。定义原发性血小板减少性紫癜，是一因血小板免疫性破坏，导致外周血中血小板减少的出血性疾病。细菌或病毒感染与此病的发病有密切的关系。80%的急性ITP患者，发病前两周左右有呼吸道感染史。感染不能直接导致ITP的发病，免疫因素的参与可能是ITP发病的重要原因，80%以上的ITP患者血小板表面可检测到血小板抗体;此外，吸附这种抗体的血小板易在肝脾(主要在脾脏)中被巨噬细胞吞噬，使血小板寿命缩短。血小板数量减少的程度与循环中抗血小板抗体量的多少成正比，即抗血小板抗体量越多，血小板减少的程度越严重。新生儿患者其母可患有同样疾病，由于抗体可通过胎盘，进入胎儿体内，导致新生儿血小板减少。以上特点都支持ITP是免疫机制引起的疾病。脾脏不但是破坏血小板的主要场所，而且是产生抗血小板抗体的主要器官，脾切除有效的患者术后抗血小板抗体滴度显着下降。血小板减少性紫癜以皮肤黏膜及内脏出血，血小板减少，骨髓巨核细胞成熟障碍，血小板生存时间缩短出现等为特征，血小板过度减少导致颅内出血是本病的致死病因。 [1]英文名称idiopathic thrombocytopenic purpura其他名称免疫性血小板减少性紫癜(immune thrombocytopenic purpura），自身免疫性血小板减少性紫癜(autoimmune thrombocytopenic purpura)临床表现播报编辑急性型多为10岁以下儿童，两性无差异。多在冬、春季节发病，病前多有病毒感染史，以上呼吸道感染、风疹、麻疹、水痘居多；也可在疫苗接种后。感染与紫癜间的潜伏期多在1～3周内。成人急性型少见，常与药物有关，病情比小儿严重。起病急骤，可有发热。主要为皮肤、粘膜出血，往往较严重，皮肤出血呈大小不等的瘀点，分布不均，以四肢为多。粘膜出血有鼻衄、牙龈出血、口腔舌粘膜血泡。常有消化道、泌尿道出血，眼结合膜下出血，少数视网膜出血。脊髓或颅内出血常见，可引起下肢麻痹或颅内高压表现，可危及生命。如果患者头痛，呕吐，要警惕颅内出血的可能。病程多为自限性，80%以上可自行缓解，平均病程4～6周。少数可迁延或数年以上转为慢性。急性型占成人ITP不到10%。慢性型占ITP的80%，多为20～50岁，女性为男性的3～4倍。起病隐袭。患者可有持续性出血或反复发作，有的表现为局部的出血倾向，如反复鼻衄或月经过多。瘀点及瘀斑可发生在任何部位的皮肤与粘膜，但以四肢远端较多。可有消化道及泌尿道出血。外伤后也可出现深部血肿。颅内出血较少见，但在急性发作时仍可发生。脾脏在深吸气时偶可触及。血小板在(10～50)×109/L之间可有不同程度自发性出血，血小板小于10×109/L常有严重出血，病人除出血症状外全身情况良好。治疗播报编辑一般治疗急性型及重症者应住院治疗，限制活动，加强护理，避免外伤。禁用阿司匹林等一切影响血小板聚集的药物，以免加重出血。止血药物对症处理也很重要，如①止血敏可降低毛细血管通透性、使血管收缩、缩短出血时间，还可加强血小板粘附功能，加速血块收缩。每次250～500mg，肌肉或静脉滴注，每次250～750mg加5%葡萄糖溶液或生理盐水，2～3次/日。②安络血，可稳定血管及其周围组织中的酸性粘多糖，使血管脆性减低。10～20mg，每日三次口服，或60～100mg，加入5%葡萄糖溶液500ml静脉滴注③抗纤溶药物，如6－氨基已酸4～6g，加入5～10%葡萄糖水250ml静脉滴注，后每次用1g维持，一日量最多不超过20g。止血芳酸，每次0.1～0.3g加5%葡萄糖液，静脉滴注，每日最大量0.6g。止血环酸0.25g，每日3～4次口服，或0.25g 静脉滴注，每日1～2次。可酌情选用。慢性型女性患者月经过多时，于月经来潮前10～14天起，每日给予肌肉注射丙酸睾丸酮50mg，至月经来潮时停用，常有较好疗效。西医治疗1、急性型ITP，多发生在儿童，与病毒感染有关。故预防病毒感染是防止复发或病情恶化的关键，平素可常冲服板蓝根冲剂预防。　2、慢性型慢性期ITP患者，应注意避免过劳和外感，因为过劳和外感是加重病情转为急性期的主要因素。　3、本病发病期间应卧床休息，密切观察，避免外伤，进食易消化食物，防止致命性出血。　4．对用常规方法治疗而长期迁延不愈的患者处理：可采用下述方法：①脾放射治疗：可使20%用激素治疗1个月无效患者的血小板增加至100×109/L以上并长期稳定；②部分脾栓塞：可使约30%用激素治疗无效患者的血小板数增加至100×109/L，维持9-67个月；③切除附脾：约15%的患者有附脾，切除后一半患者的血小板计数上升，10%一30%的病人能稳定在正常水平；④免疫抑制剂：可使约一半患者的血小板上升，但停药后易复发，且有导致骨髓抑制和诱发肿瘤的危险。联合化疗虽有半数患者的血小板稳定上升，但造成死亡的可能性似也增加。　5．本病出现致命性出血患者的紧急治疗：对有严重粘膜出血、内脏大出血以及怀疑中枢神经系统出血者，应采用静脉注射丙种球蛋白治疗，能使多数患者的血小板在短期内升到理想水平，并可同时输注血小板，以每4-6小时输6-8单位的速度为宜。尽管输入的血小板可能被立即破坏，但它能保护患者免遭灾难性出血，直到特异性治疗生效。有人观察到在很多免疫性血小板减少的患者经输注血小板后血小板计数明显增高。偶有间断输血小板无效者，改用连续输入(1-2单位/小时)可控制严重出血。血浆置换也可用于严重出血患者，但费用昂贵。急症脾切除主要适用于经用上述方法治疗失败者，但术前应输注血小板使之达到理想水平。肾上腺皮质激素急、慢性型出血较重者，应首选肾上腺皮质激素，对提升血小板及防治出血有明显效果，然而停药后，半数病例可复发，但再发再治仍有效。肾上腺皮质激素可抑制单核巨噬细胞系统的吞噬作用，从而使抗体被覆的血小板的寿命延长；改善毛细血管的渗透脆性，改善出血。常用强地松，剂量；急性型时为防止颅内出血，需用剂量较大，2～3mg/kg/d，至血小板达安全水平为止。慢性型0.5～1mg/kg/d，一般需2～3周始能显效，然后逐步减少剂量，5～10mg每日或/隔日口服，维持期4～6个月。出血较重者静脉滴注氢化可地松或地塞米松疗效好。肝功能差或长期服强地松无效者，改用强地松龙有时可以奏效。长期用药者应酌情加同化类激素（如苯丙酸诺龙）。脾切除脾切除是ITP的有效疗法之一。指征：①慢性ITP，内科积极治疗6个月无效；②肾上腺皮质激素疗效差，或需用较大剂量维持者（30～40mg/d)；③对激素或免疫抑制应用禁忌者；④51Cr标记血小板检查，若血小板主要阻留在脾脏，则脾脏有效率可达90%，若阻留在肝脏，则70%的脾切除无效。脾切除有效率可达70～90%，术后复发率9.6～22.7%。长期效果为50～60%。免疫抑制剂环磷酰胺50～150mg/d口服，一般2～6周才可奏效，缓解率30～40%,对骨髓抑制作用强。硫唑嘌呤50～150mg/d口服，缓解率40%，需长期用药。长春胺生物碱可选择性地与单核巨噬细胞的微管球蛋白结合，抑制它们的吞噬作用和C3受体功能。长春新碱（VCR）0.025mg/kg，每次1mg，或长春花碱（VLB）0.125mg/kg，每次不超过10mg，溶于500～100ml生理盐水，缓慢静滴8～12小时，每7～10天一次，3～4次为一疗程，疗效较好。免疫球蛋白作用：①抑制自身抗体的产生；②抑制单核巨噬细胞的FC受体的功能；③保护血小板免被血小板抗体附着。适应证：①并发严重出血的急性重症ITP；②慢性ITP患者手术前准备；③难治性ITP。疗效60%左右，能快速升高血小板，但不能持久。首次剂量400mg/kg静脉滴注，连续5天，维持量400mg/kg每1～6周一次。皮质激素能影响免疫球蛋白对巨噬细胞的阻断作用，不宜合用。达那唑（danazol，炔羟雄烯异恶唑）是一种合成雄激素，但其雄性作化用已被减弱。其作用可能是与恢复抑制性T细胞功能使抗体减少有关。剂量为每日口服400～800mg，疗程≥2个月，孕妇禁用，定期查肝功能。输注血小板用于有危及生命的出血患者或术前准备。6～20U/日，每输血小板2.5U（每单位相当于200ml全血所含血小板），可使血小板升高10×109/L。如先输注免疫球蛋白再输注血小板，可使血小板寿命延长。输注血小板易使受者产生同种抗体，影响以生输注效果。血浆置换适用于急性重症患者，以图在短时间内除去部分抗血小板抗体。每日交换血浆3～5单位，连续数日。慢性ITP一般无效。促血小板生成药目前尚无有效的促血小板生成药。可用肌苷200～600mg，每日三次口服；或200～600mg静脉注射或滴注，每日1～2次。氨肽素1g，每日三次口服。核苷酸100～200mg，每日三次口服。中医治疗原发性血小板减少性紫癜的病因及发病机理，迄今尚未被阐明。多认为是一种与免疫有关的疾病。中医认为原发性血小板减少性紫癜属于祖国医学血证中的"紫斑"、"肌衄"和"葡萄疫"的范围。紫斑是以血液溢出肌肤之间，皮肤出现青紫斑点或斑块为特征，并伴有鼻衄、齿衄，严重者可有呕血、便血、脑衄。早在《内经》中即对血的生理及病理有较深入的认识，并对引起出血的原因及部分血证的预后有所论述。《灵枢.百病始生》中指出："阳络伤则血外溢，血外溢则衄血；阴络伤则血内溢，血内溢则后血"。阳络伤指的是人体上半部的络脉损伤，会出现鼻衄、齿衄等；阴络伤指人体的下半部经脉受损，故出现便血。 正常的人体状态，人体内环境里的五脏六胕按照一定的规律动行。在发病诱因（这个诱因可以来自内外两方面）的刺激下，奇恒之腑的"髓"做出反应，产生一个整体观念的脏腑模式，以应付变化。这一模式以整体的方式投影到全身各个脏腑器官。迅速发生作用，并形成新的身体状态，当这一状态进一步强化了对"髓"的刺激，就形成了恶性循环。这一恶性循环在全身的持续作用，使机体发生病理变化，从而表现为全身症状， 皮肤瘀斑或瘀点，鼻衄，齿龈出血，血尿，月经过多，便血，脑出血等，还可表现出贫血的症状。 也就是我们所说的血小板减少性紫癜。血小板减少性紫癜是一种常见的出血性疾病，多发生于儿童及青年，尤以女青年为多见。临床表现为皮肤出现瘀点及瘀斑，皮肤黏膜出血，甚者鼻出血、吐血，尿血便血，内脏出血。本病属于中医"发斑"、"血证"范畴，病因：由于热毒炽盛，气不摄血，致使血妄行；或可能为肝实脾虚，肝木凌土，脾不统血而引发该病。病情长久不愈会导致脾肾阳虚或肝肾阴虚。中药治疗西医治疗原发性血小板减少性紫癜以激素治疗为主，如强的松，也采用兔疫抑制剂等治疗，严重时输血和血小板。中医治疗以凉血 解毒活血为主，兼顾益气养阴。中医治疗以髓入手，全身治疗，使病症开始好转，人体恢复正常生理机能。奇恒之腑的髓到全身各器官，损有余而补不足，修复已造成的器官损害，使病症开始好转，形成新的人体状态。由于器官损害的多样性，根据病情随症加减调整处方中药药物治疗，使治疗效果达到最佳。专家中药方剂血小板减少性紫癜是常见的出血性疾病。运用中药方剂使患者逐渐递减激素为零，生活恢复正常。 原发性血小板减少性紫癜是指血液中血小板数减少，引起皮肤、粘膜出血，严重者内脏亦有出血。其临床症状表现为自发性的皮肤瘀点、瘀斑，粘膜 和内脏出血，血小板减少及出血时间延长等。血小板减少性紫癜分为原发性和继发性两类。原发性血小板减少性紫癜，又有急性型和慢性型之分。慢性型 较常见，女性为男性的3 ～4倍 。原发性血小板减少性紫癜的病因及发病机理，迄今尚未被阐明，多认为是一种与免疫有关的疾病。一般治疗以激素为主，如 肾上腺皮质激素类，出血症状改善，血小板接近正常后，逐渐减量维持治疗。免疫抑制剂常与糖皮质激素合用。 虽然激素能升高血小板，但是激素减量或停止后，血小板又会下降；经常使用激素，对人体副作用非常大，在治疗的同时还会引起如向心性肥胖、高血压、糖尿病等疾病。其他治疗方法如 输注血小板、脾脏切除等方法治疗，虽能起到一定疗效，但因消耗快、费用高、副作用大，大多数患者难以承受。专家中药方剂在对患者治疗过程中可以使激素用量逐渐递减为零，并最终代替激素。经临床观察，总有效率为98% ，临床治愈率为76% 。慢性ITP的中医辨证大多属气虚出血，宜用养气止血法，代表方为归脾汤。功能与主治：滋阴补肾，凉血止血。用于血小板减少性紫癜等。主要用于治疗各种出血性疾病。它可以提高机体免疫力，改善骨髓造血功能，改善血管通透性、增加血管弹性，能有效阻止皮肤 及各器官出血的发生。还有很强的抗病毒作用，也用于治疗一些病毒感染 引起的疾病,如传染性软疣、尖锐湿疣、扁平疣、带状疱疹、腮腺炎 等。功效：养阴清热，凉血止血。主治：⑴出血性疾病：过敏性紫癜、血小板减少性紫癜、血小板减少症、再生障碍性贫血、白血病、血友病、鼻衄、吐血、咳血。⑵病毒性疾病：传染性软疣、寻常疣、尖锐湿疣、扁平疣、带状疱疹、腮腺炎。⑶其它：酒糟鼻、皮疹、五心烦热。注意事项：禁烟、酒、辛、辣。 禁食鱼、虾、蟹、蛋、鸡和牛奶等富含蛋白质食品。原发性血小板减少西医只能采用激素来控制症状，但是达不到根治，一但停用激素，反而会加重病情。 本病采用中药治疗可以达到康复。　中成药1.复方胎盘片 每次4片，每日3次。2.归脾丸 每次10粒，每日3次。 简便方1.生石膏30克、黄柏15克、五倍子15克、儿茶6克，浓煎漱口，每日5～10分钟。适用于紫癜兼齿衄较甚者。2.大枣50个、白茅根30克，水煎服，每日1次，分3次服汤食枣。适用于本病慢性型。食疗法：（1）藕枣胶：大枣4份、藕节1份、冰糖适量，加水煮至黏胶状，每天服适量大枣，可补血止血。（2）茅根饮：白茅根15克、花生衣30克、白糖适量，煎汤代茶，功能凉血止血。饮食疗法中医认为，本病多为血热妄行，阴虚火旺，气虚失摄，瘀血内阻所为，当以清热凉血，滋阴清热，健脾益气，化瘀止血为治。可选用下列药膳食疗方。猪皮茅根蜜饮猪皮500g，茅根60g，白蜜适量。将猪皮去毛洗净，切块备用，茅根水煎取汁，纳入猪皮煮至稠粘，调人白蜜拌匀煮沸，每剂分4次服食，每日l剂。可清热养阴，凉血止血。适用于血热妄行，皮肤紫癜，吐衄，齿衄，血色鲜红等。藕节二叶汤藕节5个，荞麦叶、枸杞叶各lOOg。将藕节、二叶洗净，水煎取汁饮服，每日l剂。可清热解毒，凉血止血。适用于热毒炽盛，迫血妄行之紫癜，吐衄，鼻衄等。水牛角汤：水牛角30g，旱莲草50g。将水牛角削片，加清水500ml煮沸2小时后，加旱莲草再煮20分钟，去渣取汁饮服，每日l剂。可清热解毒，凉血止血。适用于热迫血行之紫癜各种出血，口干舌燥，便秘尿黄，心情急躁易怒等。猪蹄爪甲地黄汤猪蹄爪甲4个，生地、白芍、丹皮各lOg。将猪蹄甲加水煮约 2小时，投入诸药再煮半小时饮服，每日1剂。可清营凉血。适用于热煎营血之各种出血。梨藕荸荠煎：鲜梨2个，鲜藕150g，荸荠lOOg，生地15g，蜂蜜适量。将梨、藕、荠去皮洗净，切块，同生地水煎取汁，兑入蜂蜜适量饮服，每日l剂。可清热养阴，凉血止血。适用于阴虚血热之各种出血。滋阴凉血膏：麦冬、天冬、生地、阿胶、电胶、鱼鳔胶、冰糖各50g，红枣100枚，黄酒20m1。将二冬、地、枣水煎，留枣去渣取汁，纳入三胶烊化，而后纳入冰糖、黄酒，慢火收膏。每次20ml，每日3次，温开水冲饮，嚼食大枣。可滋阴清热，凉血止血。适用于阴虚内热之紫癜散在，颜色紫红，下肢尤甚，时发时止等。天冬蜜饮 天冬50g(鲜者150g)，蜂蜜适量。将天冬加水3碗，煎至1碗半，兑入蜂蜜饮服，每日l剂。可滋阴凉血。适用于阴虚血热之紫癜，伴腰膝酸软，头晕耳鸣，低热盗汗，五心烦热，口干咽燥等。侧柏生地茅根汤：侧柏叶15g，生地 50g，白茅根1000g，蜂蜜适量。将三药水煎 2次，二液合并，兑人蜂蜜，煮沸饮服，每日 l剂。可滋阴清热，凉血止血。适用于阴虚血热之紫癜。黑豆芪鱼汤 黑豆60g，塘虱鱼(胡子鲶)2条，黄芪18g，调料适量。将虱鱼去两侧颈花及肠杂，洗净，黄芪布包，同黑豆加清水适量同煮至豆、随熟后，去药包，加食盐、味精等调服。可温肾健脾，益气摄血。适用于脾肾两虚之紫癜，斑色淡红，清稀不显，伴面包苍白，头晕乏力，形寒肢冷，神倦纳呆等。原发性血小板减少性紫癜是一种与自身免疫有关的疾病。表现为皮肤、黏膜出现瘀点瘀斑，或见齿龈出血、鼻衄及月经过多等，严重者可出现内脏出血。本病在临床上可分为两型：急性型多见于儿童或青年，发病突然，有畏寒，发热，继之皮肤和黏膜出现紫癫，血小板计数明显减少，骨髓中无血小板形成；慢性型多见于成年人，尤以成年妇女多见，缓解和发作交替出现，出现症状较轻，血小板计数减少，骨髓中血小板形成减少。原发性血小板减少性紫癜属中医“血证”、“虚劳”等范畴。病因及发病机制播报编辑本病的病因和发病机制尚未完全清楚。急性型多发生于急性病毒性上呼吸道感染痊愈之后，提示血小板减少与对原发感染的免疫反应间有关，可能感染后，在体内形成抗原－抗体复合物，通过其抗体分子上的FC片段与血小板上FC受体相结合。附有免疫复合物的血小板易在单核噬细胞系统内被破坏，而导致血小板减少。这一现象在体外已得到证实，故可认为是一种免疫复合体病。另一种理论认为，感染因素改变了血小板膜的结构，使其具有抗原性，致产生抗自身血小板抗体（自身免疫性疾病）。或者抗病毒抗体对血小板膜抗原有交差免疫反应。这些假说尚待证实。慢性型患者中约半数可测出血清中有抗血小板抗体，该抗体分别属于免疫球蛋白G、M、A、C3、C4等，而以IgG最多见。血小板表面亦可结合免疫球蛋白，称血小板表面相关免疫球蛋白（PAIg），一般认为PAIgG可能是真正的抗血小板抗体，通过其IgG分子上Fab片段与血小板特异性抗原结合，然后通过其FC片段与巨噬细胞受体结合，致血小板被吞噬和破坏。若血小板表面结合的IgG量多时，则可形成IgG双体，并激活补体，巨噬细胞上的FC和C3b受体起协同作用，血小板更易被破坏。PAIgG量与病情呈正相关。血小板和巨核细胞二者有共同抗原性，巨核细胞亦可直接受破坏。ITP患者产生抗血小板抗体的相关抗原，可能为血小板膜糖蛋白（GP）Ⅱb/Ⅲa。细胞免疫　在发病机制中具体作用尚不清楚。TS的功能缺陷可能在本病中起一定作用。脾脏因素 通过体内闪烁扫描技术，以放射性同位素标记之抗体作用于血小板，发现约59%的结合抗体和血小板在脾内破坏；约14%在肝内破坏，以破坏结合抗体量多的血小板为主，故后者多见于重症病例。此外，脾脏也是自身抗体合成的主要部位。雌激素的作用 雌激素对血小板生成有抑制作用，并能促进单核巨噬细胞对结合抗体血小板的吞噬作用。实验室检查播报编辑血象急性型血小板明显减少，多在20×109/L以下。出血严重时可伴贫血，白细胞可增高。偶有嗜酸性粒细胞增多。慢性者，血小板多在30～80×109/L，常见巨大畸形的血小板。骨髓象急性型 巨核细胞数正常或增多，多为幼稚型，细胞边缘光滑，无突起、胞浆少、颗粒大。慢性型，巨核细胞一般明显增多，颗粒型巨核细胞增多，但胞浆中颗粒较少，嗜碱性较强。免疫学检查目前国内外多采用直接结合试验，如核素标记、荧光标记或酶联抗血清的PAIg检测法。国内应用酶联免疫吸附试验测定ITP患者PAIgG，PAIgM和PA－C3阳性率分别为94%、35%、39%。其增高程度与血小板计数负相关。急性型时PAIgM多见。巨核表面细胞亦可查出抗血小板自身抗体。其他出血时间延长，束臂试验阳性，血块收缩不佳，血小板粘附、聚集功能减弱，51Cr或111In标记血小板测定，其寿命缩短。诊断及鉴别诊断播报编辑国内诊断标准（1）多次化验检查血小板减少；（2）脾脏不增大或仅轻度增大；（3）骨髓检查巨核细胞正常增多，有成熟障碍；（4）具备以下5点中任何一点：①强地松治疗有效；②脾功除有效；③PAIg增高；④PAC3增高；⑤血小板寿命缩短；（5）排除继发性血小板减少症。急性型须与某些严重之细菌感染，尤其是脑膜炎球菌感染；急性白血病，药物过敏及弥散性血管内凝血相鉴别。免疫性血小板减少症尚可见于红斑狼疮、结核病、结节病、甲状腺机能亢进、慢性甲状腺炎及自身免疫性贫血（Evans综合征）。诊断要点1.急性型起病急骤，出血症状严重，多见于儿童。慢性型起病缓慢，亦有明显出血倾向。2.血液检查 血小板减少，出血时间延长，血块收缩不良，毛细血管脆性试验阳性。凝血时间正常。3.骨髓象 巨核细胞增多或正常，伴成熟障碍。4.血小板表面IgG、IgM或补体增高。原发性血小板减少性紫癜的危害播报编辑1、血小板减少性紫癜患者起病急骤，可有发热。 主要为皮肤、粘膜出血，往往较严重，皮肤出血呈大小不等的瘀点，分布不均，以四肢为多。粘膜出血有鼻衄，牙龈出血，口腔舌粘膜血泡。常有消化道，泌尿道出血，眼结合膜下出血，少数视网膜出血。脊髓或颅内出血常见，可引起下肢麻痹或颅内高压表现，可危及生命。2、血小板减少性紫癜患者受到的伤害主要为皮肤、粘膜出血，往往较严重，皮肤出血呈大小不等的瘀点，分布不均，以四肢为多。粘膜出血有鼻衄，牙龈出血，口腔舌粘膜血泡。常有消化道，泌尿道出血，眼结合膜下出血，少数视网膜出血。脊髓或颅内出血常见，可引起下肢麻痹或颅内高压表现，可危及生命。病程及预后播报编辑急性型的病程短，有自愈趋势，约80%患者可以缓解。50%患者可在6周内恢复，其余的在半年内完全恢复，6～20%可转为慢性，病死率1%，多在发病1～2周时。慢性型有10～20%可以自愈，多数病程较长，发作与缓解相间隔，有的呈周期性发作。个别严重患者，血小板极度减少，有颅内出血危险，后者为本病的致死原因。病因病理播报编辑原发性血小板减少性紫癜的病因常为外感热毒或内生邪热，壅遏脉络，迫血妄行，而致血溢肌肤；或饮食劳倦，损伤心脾，气虚不能摄血所致。病理变化为外感热毒，内伏营血，络脉所伤，而血不循经，溢于脉外；或热邪传里，胃热熏蒸，以及过食辛辣酒浆，致胃中伏热，热邪扰动阴血，血液溢于肌肤，故发病急，皮肤、黏膜广泛出血。亦可由劳倦内伤，反复出血，损伤心脾，气随血耗，血失统摄测溢于络脉之外；阴虚火旺，阴虚则络脉失养，火旺则脉络受伤，虚火内动，扰乱营血，血随火动，离经妄行，致血溢脉外，一般出血较轻，除皮肤、黏膜出血外，无广泛性出血。辩证分型播报编辑血热妄行症状皮肤出现紫色瘀点或瘀斑，或伴有鼻衄、齿衄、便血、尿血，或发热，口干，便秘。舌红苔黄，脉弦数。多见于本病早期或急性型。证候分析：热毒内伏营血，化火动血，灼伤络脉，迫血妄行，溢出常道，故出现皮肤紫癜，或伴其他血证；热毒乃火热之邪，内热郁蒸，故发热；热盛伤津，故口干、便秘；舌红苔黄，脉弦数为实热之征象。阴虚火旺症状紫癜较多，时发时止，常伴鼻衄、齿衄或月经过多，颧红，心烦，口渴，手足心热，或潮热，盗汗。舌质红绛，少苦，脉细数。多见于本病慢性型。证候分析：阴虚火旺，虚火伤及脉络，血溢脉外，故见皮肤紫癜，或他处出血，如鼻衄、齿衄或月经过多等；虚火上炎，故颧红潮热；虚火扰心，则心烦；阴伤津亏，故口渴；阴火迫津外泄故盗汗；舌红绛少苔，脉细数均为阴虚火旺之征。气不摄血症状久病不愈，反复出现紫癜，神疲乏力，头晕目眩，面色苍白或萎黄，食欲不振。舌质淡胖，脉细弱。多见于本病慢性型。证候分析：久病不愈，脾气亏虚，失于统摄，血不循经，故反复出现紫癫；气血亏耗，则见神疲乏力，头晕目眩，面色苍白或萎黄；脾虚不能运化水谷，故食欲不振；舌淡胖，脉细弱为气血亏虚之象。分型治疗播报编辑1.血热妄行治则：清热解毒，凉血止血。方药：清营汤合十灰散加减。犀角尖3克生地30克玄参15克竹叶心12克麦冬15克丹参30克黄连6克金银花15克连翘15克大蓟15克小蓟15克荷叶6克侧柏叶12克白茅根30克茜草根15克棕榈皮12克丹皮15克随症加减：高热，出血广泛严重者，加生石膏30克、龙胆草9克、紫草9克，以泻火清热；腹痛、便血者，加白芍12克、甘草9克、五灵脂9克（包煎）、蒲黄9克、木香6克、地偷12克，以缓急止痛，活血理气止血。2.阴虚火旺治则：滋阴降火，宁络止血。方药：茜根散为主。茜草根15克黄芩9克阿胶9克侧柏叶12克生地15克甘草6克随症加减：潮热盗汗明显者，加玄参12克、龟版9克、女贞子12克、旱莲草12克，以加强滋阴之力。3.气不摄血治则：补气摄血。方药：归脾汤加减。黄芪15克当归10克白术10克党参12克木香6克远志3克酸枣仁9克龙眼肉9克炙甘草6克棕榈炭9克仙鹤草30克大枣7枚随症加减：肾虚腰膝酸软，遗精阳痿或月经不调者，加山茱萸 9克、菟丝子 12克、川断 12克、鹿角胶9克，以补肾填精。注意事项播报编辑1.要及时明确诊断，对症治疗，防止内脏出血，危及生命。2.本病急性型以西医治疗为主，慢性型可配合中医治疗，能提高疗效。3.饮食当以新鲜蔬菜、水果及营养消化食物为主，忌辛辣刺激食物。4.饮食以高蛋白、高维生素及易消化饮食为主，避免进食粗硬食物及油炸或有刺激的食物，以免易形成口腔血泡乃至诱发消化道出血。5.多食含维生素C、P的食物，多食花生、红枣、核桃、桂圆、扁豆等益气养血之品。禁酒。6.慢性期多为脾肾不足、气血两亏，饮食调理上宜偏温补，如鸡、狗、牛、羊和猪肉等，鳝鱼、鲫鱼、鲤鱼、乌贼鱼等以及牛奶、禽蛋、各种新鲜蔬菜及水果等。 [2]护理预防播报编辑1.积极参加体育活动，增强体质，提高抗病能力。 2.要注意预防呼吸道感染、麻疹、水痘、风疹及肝炎等疾病，否则易于诱发或加重病情。 3.急性期或出血量多时，要卧床休息，限制患儿活动，消除其恐惧紧张心理。 4.避免外伤跌仆碰撞，以免引起出血。 5.血小板计数低于20×109/L时，要密切观察病情变化，防止各种创伤与颅内出血。 6.饮食宜清淡，富于营养，易于消化。呕血、便血者应进半流饮食，忌硬食及粗纤维食物。忌辛辣刺激食物。患儿平素可多吃带衣花生仁、红枣等食物。 [3]新手上路成长任务编辑入门编辑规则本人编辑我有疑问内容质疑在线客服官方贴吧意见反馈投诉建议举报不良信息未通过词条申诉投诉侵权信息封禁查询与解封©2024 Baidu 使用百度前必读 | 百科协议 | 隐私政策 | 百度百科合作平台 | 京ICP证030173号 京公网安备110000020000

免疫性血小板减少性紫癜（ITP） - 血液病学及肿瘤病学 - MSD诊疗手册专业版

免疫性血小板减少性紫癜（ITP） - 血液病学及肿瘤病学 - MSD诊疗手册专业版

默沙东 诊疗手册

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血液病学及肿瘤病学

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血小板减少和血小板功能不全

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免疫性血小板减少性紫癜（ITP）

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在该主题中

本章节的其他主题

血小板疾病的概述

获得性血小板功能异常

遗传性内源性血小板疾病

免疫性血小板减少性紫癜（ITP）

脾脏阻留引起的血小板减少

血小板减少症：其他原因

溶血尿毒综合征（HUS）

血栓性血小板减少性紫癜（TTP）

血管性血友病

免疫性血小板减少性紫癜（ITP）

（特发性血小板减少性紫癜；免疫性血小板减少性紫癜）

作者：

David J. Kuter

, MD, DPhil, Harvard Medical School

医学审查 6月 2022

看法 进行患者培训

症状和体征

诊断

预后

治疗

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瘀点（硬腭）

免疫性血小板减少性紫癜（ITP）

瘀斑

免疫性血小板减少的瘀斑

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免疫性血小板减少症 (ITP) 是一种出血性疾病，通常没有贫血或白细胞减少症。 成年患者通常为慢性，而儿童常是急性和自限性。在没有其他原发疾病时，脾脏大小是正常的。诊断通常是临床的，基于排除血小板减少症的其他原因（例如，HIV感染和丙型肝炎感染）。治疗包括糖皮质激素、脾切除、免疫抑制剂和血小板生成素受体激动剂或者脾酪氨酸激酶抑制剂fostamatinib。对于致命的出血，可单独或联合使用血小板输注、静脉滴注糖皮质激素、静脉给予抗免疫球蛋白G或静脉注射免疫球蛋白进行治疗。 (参见 于血小板疾病的概述 血小板疾病的概述 血小板是在凝血系统中起作用的循环细胞碎片。血小板生成素通过刺激骨髓巨核细胞的产生来控制循环中血小板的数量，反过来影响血小板从胞质的脱落。血小板生成素在肝脏中以恒定速率产生，其循环水平取决于循环血小板和骨髓巨核细胞的结合量以及循环血小板的清除程度。血小板在外周血中可存活7～10天。大约1/3的血小板总... Common.TooltipReadMore 。） 免疫性血小板减少性紫癜通常由于针对结构性血小板抗原的自身抗体形成所致。这些抗血小板抗体导致血小板破坏增加，通常在脾脏中，并抑制血小板产生和从巨核细胞释放。在儿童ITP中，自身抗体可能由病毒抗原所诱发。虽然在一些国家（例如日本，意大利），成人的触发因素仍未知，但ITP与幽门螺杆菌 Helicobacter pylori 感染有关，且在治疗感染后，ITP可缓解 (1 参考文献 免疫性血小板减少症 (ITP) 是一种出血性疾病，通常没有贫血或白细胞减少症。 成年患者通常为慢性，而儿童常是急性和自限性。在没有其他原发疾病时，脾脏大小是正常的。诊断通常是临床的，基于排除血小板减少症的其他原因（例如，HIV感染和丙型肝炎感染）。治疗包括糖皮质激素、脾切除、免疫抑制剂和血小板生成素受体激动剂或者脾酪氨酸激酶抑制剂fostamatinib。对于致命的出血，可单独或联合使用血小板输注、静脉滴注糖皮质激素、静脉给予抗免疫球蛋... Common.TooltipReadMore )。新冠肺炎感染很少引起ITP，但新冠肺炎疫苗接种可能会加重2%至12%的ITP患者的血小板减少症。 妊娠往往会加重ITP 妊娠期免疫性血小板减少 自身免疫疾病在女性中是普通疾病的5倍，并且育龄年龄期间，发病率处于峰值。 因此，这些疾病在孕妇中普遍发生。 抗磷脂综合征（APS）是一种自身免疫性疾病，易导致患者血栓形成，并使怀孕期间风险增加 胎儿死亡 妊娠期高血压 子痫前期 Common.TooltipReadMore ，并增加产妇发病的风险。 参考文献 1.Kuter DJ: The treatment of immune thrombocytopenia (ITP)—focus on thrombopoietin receptor agonists.Annals of Blood Volume 6 March 2021 ITP的症状和体征 虽然通常无症状，仅通过常规检测中的低血小板计数来识别，但是当出现免疫性血小板减少的症状和体征时，则表现为 瘀点 紫癜和/或瘀斑 粘膜出血 月经出血增多 严重的消化道出血和血尿不常见。脾脏大小正常，但合并病毒感染或 自身免疫性溶血性贫血 自体免疫性溶血性贫血 自身免疫性溶血性贫血是抗体与红细胞在温度≥37°C（温抗体型溶血性贫血）或<37°C时（冷抗体型溶血性贫血）发生反应而产生的。溶血是血管外的。直接抗人球蛋白（直接Coombs）试验可建立诊断并可提示病因。治疗取决于病因，可能包括皮质类固醇、脾切除术、IV 免疫球蛋白、免疫抑制剂、避免诱发因素（如感冒）和停药。 也见于 溶血性贫血的概述 自身免疫性溶血性贫血是由 红细胞外在异常所致... Common.TooltipReadMore 时是增大的（Evans综合征）。与其他血小板破坏增加的疾病一样，ITP也与血栓形成风险的增加有关。 免疫性血小板减少的表现 瘀点（硬腭） 瘀点的特征是在该患者的上颚上看到的小红点。 DR P.MARAZZI/SCIENCE PHOTO LIBRARY 免疫性血小板减少性紫癜（ITP） 经出版商许可。From Deitcher S.In Atlas of Clinical Hematology.Edited by JO Armitage.Philadelphia, Current Medicine, 2004. 瘀斑 瘀斑是在该患者腿上看到的大的紫色瘀伤。 DR P.MARAZZI/SCIENCE PHOTO LIBRARY 免疫性血小板减少的瘀斑 经出版商许可。摘自 Deitcher S.In Atlas of Clinical Hematology.Edited by JO Armitage.Philadelphia, Current Medicine, 2004. ITP的诊断 带血小板计数的全血细胞计数（CBC）外周血涂片 很少需要骨髓穿刺 除外其他血小板减少性疾病 孤立性血小板减少患者（即，除外的全血细胞计数和外周血涂片均正常）需考虑免疫性血小板减少。因免疫性血小板减少症(ITP)的表现是非特异的，其他引起 单纯性血小板减少的原因 血小板减少症 血小板是在凝血系统中起作用的循环细胞碎片。血小板生成素通过刺激骨髓巨核细胞的产生来控制循环中血小板的数量，反过来影响血小板从胞质的脱落。血小板生成素在肝脏中以恒定速率产生，其循环水平取决于循环血小板和骨髓巨核细胞的结合量以及循环血小板的清除程度。血小板在外周血中可存活7～10天。大约1/3的血小板总... Common.TooltipReadMore （如，药物、酒精、淋巴增殖性疾病、其他自身免疫性疾病、病毒感染）需要通过临床评估和适当的检查加以排除。 患者通常需要进行凝血功能检查、肝功能检查、HIV和丙型肝炎的检查。 必须检查外周血涂片以评估血小板大小和粒度，并帮助排除血小板减少的其他主要原因，例如 血栓性血小板减少性紫癜 血栓性血小板减少性紫癜（TTP） 血栓性血小板减少性紫癜（TTP）是一种急性暴发性疾病，以血小板减少和微血管病性溶血性贫血为主要特征。其他表现可能包括意识改变，有时发生肾衰竭。诊断需要明确特征性的实验室检查异常，包括直接抗球蛋白试验阴性的溶血性贫血、ADAMTS13水平下降。 治疗是血浆置换、皮质类固醇、利妥昔单抗，很少使用卡普拉珠单抗。 也见于 血小板疾病的概述 血栓性血小板减少性紫癜（类似于 溶血性尿毒症综合征... Common.TooltipReadMore （TTP）、遗传性血小板减少症和 白血病 白血病的概述 白血病是一种恶性疾病，涉及未成熟或异常白细胞过度产生，最终抑制了正常血细胞的产生并导致了与血细胞减少相关的症状。 虽然有时会涉及到自我更新能力有限的定向干细胞，但恶性转化常发生在多能干细胞水平上。异常增殖、克隆性扩增、异常分化以及细胞凋亡减少（程序性细胞死亡）导致恶性细胞取代了正常的血液成分。... Common.TooltipReadMore 。 在某些情况下，检测抗血小板抗体可能有助于诊断 (1 诊断参考文献 免疫性血小板减少症 (ITP) 是一种出血性疾病，通常没有贫血或白细胞减少症。 成年患者通常为慢性，而儿童常是急性和自限性。在没有其他原发疾病时，脾脏大小是正常的。诊断通常是临床的，基于排除血小板减少症的其他原因（例如，HIV感染和丙型肝炎感染）。治疗包括糖皮质激素、脾切除、免疫抑制剂和血小板生成素受体激动剂或者脾酪氨酸激酶抑制剂fostamatinib。对于致命的出血，可单独或联合使用血小板输注、静脉滴注糖皮质激素、静脉给予抗免疫球蛋... Common.TooltipReadMore )。血小板计数<50,000 cl="" (<="" 50="" ×="">9/L)时，ITP中未成熟的血小板分数可能会升高。 一般不需要进行骨髓检查以明确诊断，但如果血常规或血涂片发现除血小板减少以外的异常，或临床症状不典型，或患者对标准治疗（如，皮质类固醇）无效，则需要进行骨髓检查。在ITP患者中，骨髓检查可发现巨核细胞的数量正常或增多，此外其他骨髓检查的指标均正常。 诊断参考文献 1.Al-Samkari H, Rosovsky RP, Karp Leaf RS: A modern reassessment of glycoprotein-specific direct platelet autoantibody testing in immune thrombocytopenia.Blood Adv 4(1):9–18, 2020. doi: 10.1182/bloodadvances.2019000868 ITP 的预后 儿童患者通常可自愈，即使是严重的血小板减少，也能在数周到数月内痊愈。 在 成年人， 自发缓解发生率低于 10%。完成初步治疗后，约三分之一的患者病情缓解。高达75%的患者在5年内得到改善（1 预后参考 免疫性血小板减少症 (ITP) 是一种出血性疾病，通常没有贫血或白细胞减少症。 成年患者通常为慢性，而儿童常是急性和自限性。在没有其他原发疾病时，脾脏大小是正常的。诊断通常是临床的，基于排除血小板减少症的其他原因（例如，HIV感染和丙型肝炎感染）。治疗包括糖皮质激素、脾切除、免疫抑制剂和血小板生成素受体激动剂或者脾酪氨酸激酶抑制剂fostamatinib。对于致命的出血，可单独或联合使用血小板输注、静脉滴注糖皮质激素、静脉给予抗免疫球蛋... Common.TooltipReadMore )。然而，许多患者有轻度及稳定的病程（即，血小板计数>30,000/mcL [> 30 × 109/L]) 伴少量或无出血；他们常通过自动血小板计数发现，现在常规使用全血细胞计数检查。 预后参考 1.Sailer T, Lechner K, Panzer S, et al: The course of severe autoimmune thrombocytopenia in patients not undergoing splenectomy.Haematologica 91:1041–1045, 2006. ITP的治疗 口服糖皮质激素 静注免疫球蛋白（IVIG） 静注抗-D免疫球蛋白 有时需行脾脏切除术 血小板生成素受体激动剂（TPO-RA） 利妥昔单抗 Fostamatinib 其他免疫抑制剂 对于严重的出血，可静脉输注免疫球蛋白、静脉输注抗-D免疫球蛋白、静注糖皮质激素和/或血小板输注。 2019 年指南现已发布 (1, 2) 治疗参考文献 免疫性血小板减少症 (ITP) 是一种出血性疾病，通常没有贫血或白细胞减少症。 成年患者通常为慢性，而儿童常是急性和自限性。在没有其他原发疾病时，脾脏大小是正常的。诊断通常是临床的，基于排除血小板减少症的其他原因（例如，HIV感染和丙型肝炎感染）。治疗包括糖皮质激素、脾切除、免疫抑制剂和血小板生成素受体激动剂或者脾酪氨酸激酶抑制剂fostamatinib。对于致命的出血，可单独或联合使用血小板输注、静脉滴注糖皮质激素、静脉给予抗免疫球蛋... Common.TooltipReadMore 。 血小板计数> 30,000/mcL (> 30 × 109/L) 且无出血的无症状患者不需要治疗，可以对其进行监测。 新诊断为ITP并伴有出血且血小板计数<30,000><>9/L）的成年人通常初始就口服糖皮质激素（如泼尼松1mg/kg口服，每天一次）。另一种可能同样有效的糖皮质激素治疗方案是地塞米松，40mg/天，口服，连用4天。在大多数患者中，血小板计数会在2到5天内增加。但是，某些患者起效可能需要2至4周。当见效后皮质类固醇逐渐减量时，大多数成年患者会复发。重复给予糖皮质激素治疗可能是有效的，但会增加不良反应的风险。糖皮质激素通常不应连续使用超过最初的几个月；也可尝试其他药物以避免切除脾脏。如果药物治疗有效，大多数指南建议在考虑脾切除术之前至少持续一年。 对于拔牙、分娩、手术或其他侵入性检查的患者，需要口服糖皮质激素、静脉注射免疫球蛋白或抗D-免疫球蛋白以短暂升高血小板数量。血小板生成素受体激动剂（TPO-RA，例如 romiplostim、eltrombopag、avatrombopag）也可在侵入性操作之前使用，但不应用于分娩。静脉注射用免疫球蛋白或静脉用抗D免疫球蛋白也可用于 免疫性血小板减少症 ITP中的致死性出血 免疫性血小板减少症 (ITP) 是一种出血性疾病，通常没有贫血或白细胞减少症。 成年患者通常为慢性，而儿童常是急性和自限性。在没有其他原发疾病时，脾脏大小是正常的。诊断通常是临床的，基于排除血小板减少症的其他原因（例如，HIV感染和丙型肝炎感染）。治疗包括糖皮质激素、脾切除、免疫抑制剂和血小板生成素受体激动剂或者脾酪氨酸激酶抑制剂fostamatinib。对于致命的出血，可单独或联合使用血小板输注、静脉滴注糖皮质激素、静脉给予抗免疫球蛋... Common.TooltipReadMore （ITP）的致命性出血，但很少用于长期治疗，因为其作用持续时间可能只有几天到几周。 对于2/3的糖皮质激素治疗后复发的患者，脾脏切除可获得完全缓解。脾切除术通常用于无法用药物控制出血的严重血小板减少症（如，<15,000 cl="" [<="" 15="" ×="">9/L])）患者或那些疾病在 12 个月后仍然存在的人。如果二线药物治疗可以控制血小板减少，脾切除术往往没有必要(1, 2 治疗参考文献 免疫性血小板减少症 (ITP) 是一种出血性疾病，通常没有贫血或白细胞减少症。 成年患者通常为慢性，而儿童常是急性和自限性。在没有其他原发疾病时，脾脏大小是正常的。诊断通常是临床的，基于排除血小板减少症的其他原因（例如，HIV感染和丙型肝炎感染）。治疗包括糖皮质激素、脾切除、免疫抑制剂和血小板生成素受体激动剂或者脾酪氨酸激酶抑制剂fostamatinib。对于致命的出血，可单独或联合使用血小板输注、静脉滴注糖皮质激素、静脉给予抗免疫球蛋... Common.TooltipReadMore )。脾切除会导致血栓形成和感染的风险增加（特别是含荚膜的细菌，如肺炎球菌）；故患者需要接种针对肺炎链球菌Streptococcus pneumoniae、流感嗜血杆菌Haemophilus influenzae和脑膜炎奈瑟菌Neisseria meningitidis的疫苗 （最好在手术前 > 2周）。 二线治疗方案 二线治疗方案 可供以下免疫性血小板减少患者进行选择 有意向延缓脾切除，希望自发缓解 没有脾脏切除的指征或拒绝脾切除 脾切除术无效的患者 这样的患者血小板计数通常<10,000～20,000/mcL (< 10～20 × 109/L), （因此有较高的出血风险）。二线治疗方案包括血小板生成素受体激动剂（TPO-RAs）、利妥昔单抗，福坦替尼或其他免疫抑制剂。 血小板生成素受体激动剂（如每周皮下注射一次1至10 mcg/kg的罗米司亭、每天口服一次25至75 mg的艾曲波帕和每天一次20 mg的阿伐曲泊帕）的有效率> 85%。血小板生成素受体激动剂通常需要持续给药以维持血小板计数 > 50,000/mcL（>50×109/L），但数据表明，三分之一的成年人将在1年后获得无治疗缓解，2年后超过50%。 利妥昔单抗（375 mg/m2 静注，每周1次，共4周）的有效率为57％，但只有21％的成年患者在5年后仍处在缓解状态（3 治疗参考文献 免疫性血小板减少症 (ITP) 是一种出血性疾病，通常没有贫血或白细胞减少症。 成年患者通常为慢性，而儿童常是急性和自限性。在没有其他原发疾病时，脾脏大小是正常的。诊断通常是临床的，基于排除血小板减少症的其他原因（例如，HIV感染和丙型肝炎感染）。治疗包括糖皮质激素、脾切除、免疫抑制剂和血小板生成素受体激动剂或者脾酪氨酸激酶抑制剂fostamatinib。对于致命的出血，可单独或联合使用血小板输注、静脉滴注糖皮质激素、静脉给予抗免疫球蛋... Common.TooltipReadMore )。 交替给药方案也是有效的（例如，间隔 2 周给予 1000 mg IV 的剂量）。利妥昔单抗可能会影响6至12个月内接种疫苗的反应能力。 Fostamatinib是一种脾酪氨酸激酶抑制剂，据报道其应答缓解率为18％。 剂量为100mg po bid，如果血小板计数未增加至> 50,000/mcL (> 50 × 109/L— 4 治疗参考文献 免疫性血小板减少症 (ITP) 是一种出血性疾病，通常没有贫血或白细胞减少症。 成年患者通常为慢性，而儿童常是急性和自限性。在没有其他原发疾病时，脾脏大小是正常的。诊断通常是临床的，基于排除血小板减少症的其他原因（例如，HIV感染和丙型肝炎感染）。治疗包括糖皮质激素、脾切除、免疫抑制剂和血小板生成素受体激动剂或者脾酪氨酸激酶抑制剂fostamatinib。对于致命的出血，可单独或联合使用血小板输注、静脉滴注糖皮质激素、静脉给予抗免疫球蛋... Common.TooltipReadMore )，则在1个月后增加至150mg bid。 对其他药物治疗无效，并有严重的，有症状的血小板减少的患者，可使用更强效的免疫抑制剂，如环磷酰胺、环孢素、 麦考酚酯和硫唑嘌呤。 ITP中的致死性出血 患有免疫性血小板减少症和危及生命的出血的儿童或成人，静脉用免疫球蛋白，1g/kg，每天一次，应用1~2天，或在Rh阳性患者中，单用抗D-免疫球蛋白 75mcg/Kg，可快速抑制单核巨噬细胞。静注抗D免疫球蛋白仅对未行脾切除的患者有效，并可能伴有严重的并发症，如严重溶血和 弥散性血管内凝血 弥散性血管内凝血（DIC） 弥散性血管内凝血（DIC）的发生涉及到血液循环中产生了异常大量的凝血酶和纤维蛋白。在这个过程中，出现血小板聚集增加和凝血因子的消耗。慢性进展性DIC（病程数周或数月）主要引起静脉血栓和栓塞的表现；急性进展性DIC（病程数小时或数天）主要引起出血。严重急性进展性DIC可根据血小板减少、部分凝血酶时间和凝血酶原时间延长、血浆d二聚体（或血清纤维蛋白降解产物）增加以及血浆纤维蛋白原下降加以诊断。治疗包括纠正病因，以及血小板、凝血因子（新鲜冰冻... Common.TooltipReadMore 。该治疗通常可在2～4天内增加血小板数量，但仅可维持2～4周。 大剂量甲泼尼松（1g 静脉注射，每天一次，共3天）与静注免疫球蛋白或静注抗D免疫球蛋白相比更容易管理，但效果可能欠佳。伴有ITP和致命性出血的患者也应接受血小板输注。通常不会预防性地输注血小板。 长春新碱（1.4 mg/m2;最大剂量2 mg）也可用于紧急情况下 但重复给药可能产生神经病变。 早期使用 TPO-RA 与上述疗法联合使用也可能有效（5、6 治疗参考文献 免疫性血小板减少症 (ITP) 是一种出血性疾病，通常没有贫血或白细胞减少症。 成年患者通常为慢性，而儿童常是急性和自限性。在没有其他原发疾病时，脾脏大小是正常的。诊断通常是临床的，基于排除血小板减少症的其他原因（例如，HIV感染和丙型肝炎感染）。治疗包括糖皮质激素、脾切除、免疫抑制剂和血小板生成素受体激动剂或者脾酪氨酸激酶抑制剂fostamatinib。对于致命的出血，可单独或联合使用血小板输注、静脉滴注糖皮质激素、静脉给予抗免疫球蛋... Common.TooltipReadMore ）。 儿童ITP的治疗 儿童免疫性血小板减少患者通常给予支持治疗，因为大多数患儿可自发恢复。即使血小板减少长达数月或数年，大多数儿童也可自行缓解。如果出现黏膜出血，可给予糖皮质激素或静注免疫球蛋白。但使用糖皮质激素和IVIG是有争议的，因为血小板计数增加可能不会改善临床结局。儿童很少行脾切除术。然而，如果血小板减少症严重并伴有>6个月的症状，可考虑促血小板生成素受体激动剂（罗米司亭、艾曲波帕）。 治疗参考文献 1.Neunert C, Terrell DR, Arnold DM, et al: American Society of Hematology 2019 guidelines for immune thrombocytopenia.Blood Adv 3(23):3829–3866, 2019.doi: 10.1182/bloodadvances.2019000966 2.Provan D, Arnold DM, Bussel JB, et al: Updated international consensus report on the investigation and management of primary immune thrombocytopenia.Blood Adv 3(22):3780–3817, 2019.doi: 10.1182/bloodadvances.2019000812 3.Patel VL, Mahevas M, Lee SY, et al: Outcomes 5 years after response to rituximab therapy in children and adults with immune thrombocytopenia.Blood 119:5989–5995, 2012.doi: 10.1182/blood-2011-11-393975 4.Bussel J, Arnold DM, Grossbard E, et al: Fostamatinib for the treatment of adult persistent and chronic immune thrombocytopenia: Results of two phase 3, randomized, placebo‐controlled trials.Am J Hematol 93: 921–930, 2018.doi: 10.1002/ajh.25125 5.Kuter DJ, Tarantino MD, Lawrence T: Clinical overview and practical considerations for optimizing romiplostim therapy in patients with immune thrombocytopenia.Blood Rev 49:100811, 2021. doi: 10.1016/j.blre.2021.100811 6.Lozano ML, Godeau B, Grainger J, et al: Romiplostim in adults with newly diagnosed or persistent immune thrombocytopenia.Expert Rev Hematol 13(12):1319–1332, 2020.doi: 10.1080/17474086.2020.1850253 关键点 在免疫性血小板减少症（ITP）中，免疫系统破坏了循环中的血小板，同时攻击骨髓巨核细胞，从而减少血小板生成。 需要排除其他引起单纯性血小板减少的原因（如，药物、酒精、淋巴组织增殖性疾病、其他自身免疫性疾病、病毒感染）。 儿童常可自发缓解；而成人自发缓解可能发生在第一年，但比儿童少(约33%)。 糖皮质激素（有时静注免疫球蛋白（IVIG）或静脉注射抗D免疫球蛋白）是出血或严重血小板减少症的一线治疗方案。 骨髓活检通常不需要，除非存在其他有关红细胞或白细胞异常的情况，或者考虑进行脾切除术的患者对皮质类固醇或IVIG的标准治疗没有反应。 血小板生成素受体激动剂对维持>85%的成年人的安全血小板计数非常有效。 新冠肺炎感染很少引起ITP，但新冠肺炎疫苗接种可能会加重2%至12%的ITP患者的血小板减少症。 脾切除术往往是有效的，但常用于药物治疗无效或疾病持续12个月的患者。 血小板输注仅用于危及生命的出血。

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Immune thrombocytopenia (ITP) - Symptoms and causes - Mayo Clinic

Immune thrombocytopenia (ITP) - Symptoms and causes - Mayo Clinic

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Immune thrombocytopenia (ITP)

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OverviewImmune thrombocytopenia (ITP) is an illness that can lead to bruising and bleeding. Low levels of the cells that help blood clot, also known as platelets, most often cause the bleeding.

Once known as idiopathic thrombocytopenic purpura, ITP can cause purple bruises. It also can cause tiny reddish-purple dots on the skin that look like a rash.

Children can get ITP after a virus. They most often get better without treatment. In adults, the illness often lasts months or years.

People with ITP who aren't bleeding and whose platelet count isn't too low might not need treatment. For worse symptoms, treatment might include medicines to raise platelet count or surgery to remove the spleen.Products & ServicesA Book: Mayo Clinic Family Health Book, 5th EditionNewsletter: Mayo Clinic Health Letter — Digital EditionShow more products from Mayo Clinic

Symptoms

Petechiae

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Petechiae

Petechiae

Bleeding into the skin looks like tiny reddish-purple spots, also known as petechiae. Petechiae might look like a rash. Here they appear on a leg (A) and on the stomach area (B).

Immune thrombocytopenia might not have symptoms. When symptoms occur, they might include:

Easy bruising.

Bleeding into the skin that looks like tiny reddish-purple spots, also known as petechiae. The spots mostly show up on the lower legs. They look like a rash.

Bleeding into the skin that's larger than petechiae, also known as purpura.

Bleeding from the gums or nose.

Blood in urine or stools.

Really heavy menstrual flow.

When to see a doctorMake an appointment with your health care provider if you or your child has symptoms that worry you. Bleeding that won't stop is a medical emergency. Seek help right away if you or your child has bleeding that the usual first aid efforts can't control. These include applying pressure to the area.

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CausesImmune thrombocytopenia usually happens when the immune system makes a mistake. It attacks and destroys the cells that help blood clot, also known as platelets.

In adults, an infection with HIV, hepatitis or the bacteria that causes stomach ulcers, known as H. pylori, can cause ITP. In most children with ITP, the disorder follows a virus, such as the mumps or the flu.

Risk factorsITP is more common among young women. The risk appears to be higher in people who also have other diseases in which the immune system attacks healthy tissues, such as rheumatoid arthritis or lupus.

ComplicationsRarely, immune thrombocytopenia causes bleeding into the brain. This can be fatal.

Someone who's pregnant with a low platelet count or who's bleeding has a greater risk of heavy bleeding during delivery. A health care provider might suggest treatment to keep the platelet count even.

ITP doesn't usually affect the fetus. However, the baby's platelet count should be tested soon after birth.

By Mayo Clinic Staff

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Living with immune thrombocytopenia (itp)?

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Show references

Platelet disorders: Immune thrombocytopenia (ITP). National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health-topics/immune-thrombocytopenia. Accessed Jan. 3, 2023.

Ferri FF. Immune thrombocytopenic purpura. In: Ferri's Clinical Advisor 2023. Elsevier; 2023. https://www.clinicalkey.com. Accessed Jan. 3. 2023.

Arnold DM, et al. Initial treatment of immune thrombocytopenia (ITP) in adults. https://www.uptodate.com/contents/search. Accessed Jan. 3, 2023.

AskMayoExpert. Immune thrombocytopenia (adult). Mayo Clinic; 2022.

Ami TR. Allscripts EPSi. Mayo Clinic. Feb. 1, 2023.

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免疫性血小板减少症 (ITP) - 症状与病因 - 妙佑医疗国际

免疫性血小板减少症 (ITP) - 症状与病因 - 妙佑医疗国际

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概述免疫性血小板减少症（ITP）是一种可导致瘀伤和出血的疾病。帮助血液凝固的细胞（也称为血小板）的水平过低，是引起出血的最常见原因。

ITP 原称为特发性血小板减少性紫癜，可引起发紫的淤伤。这种疾病也可以引起看起来像皮疹的微小红紫色小点。

儿童在感染病毒后可能会患上 ITP。大多数时候，他们不需要治疗就会好转。在成年人中，这种疾病常常持续数月或数年。

未出血且血小板计数不太低的 ITP 患者可能不需要治疗。如果症状更为严重，治疗方法可能包括提高血小板计数的药物或切除脾脏的手术。产品与服务书籍：《妙佑医疗国际家庭健康手册》第 5 版简报：妙佑医疗国际卫生来信 — 数字版显示妙佑医疗国际的更多产品

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皮下出血看起来像紫红色小斑点，也称为瘀点。瘀点的外观可能与皮疹相似。此处图例中瘀点分别位于腿部（A）和腹部（B）。

免疫性血小板减少症可能没有症状。如果出现症状，则可能包括：

容易瘀伤。

紫红色小斑点样皮下出血（瘀点）。斑点大多出现在小腿上，外观与皮疹类似。

比瘀点大的皮下出血（紫癜）。

牙龈或鼻出血。

尿液含血或便血。

月经量过多。

何时应就诊如果您或您的孩子有任何令您担忧的症状，请与医务人员约诊。出血不止是医疗紧急事件。如果您或您的孩子发生出血且无法通过常规急救措施控制，请立即寻求帮助。这些措施包括对出血部位施加压力。

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病因免疫性血小板减少症通常发生于免疫系统出现错误时。免疫系统攻击并破坏帮助血液凝结的细胞（血小板）。

对于成人，ITP 可能由 HIV 感染、肝炎或幽门螺杆菌（导致胃溃疡的细菌）引发。对于大多数 ITP 儿童患者，该疾病常伴随病毒性疾病，例如流行性腮腺炎或流感。

风险因素ITP 在年轻女性中更为常见。如果患有类风湿关节炎或狼疮等其他免疫系统攻击健康组织的疾病，则患上此病的风险可能会升高。

并发症在极少数情况下，免疫性血小板减少症可能会导致脑出血。这可能致命。

怀孕期间血小板计数低或出血的孕妇在分娩时大出血的风险更高。医务人员可能会建议采取治疗措施来维持血小板计数平稳。

ITP 通常不会影响胎儿。然而，宝宝出生后应尽快进行血小板计数检测。

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Living with 免疫性血小板减少症 (itp)?

Connect with others like you for support and answers to your questions in the Blood Cancers & Disorders support group on Mayo Clinic Connect, a patient community.

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显示参考文献

Platelet disorders: Immune thrombocytopenia (ITP). National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health-topics/immune-thrombocytopenia. Accessed Jan. 3, 2023.

Ferri FF. Immune thrombocytopenic purpura. In: Ferri's Clinical Advisor 2023. Elsevier; 2023. https://www.clinicalkey.com. Accessed Jan. 3. 2023.

Arnold DM, et al. Initial treatment of immune thrombocytopenia (ITP) in adults. https://www.uptodate.com/contents/search. Accessed Jan. 3, 2023.

AskMayoExpert. Immune thrombocytopenia (adult). Mayo Clinic; 2022.

Ami TR. Allscripts EPSi. Mayo Clinic. Feb. 1, 2023.

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什么是免疫性血小板减少症 (ITP)？ 血小板 血小板 血液的主要成分包括 血浆 红细胞 白细胞 血小板 阅读更多 就是凝血细胞，它们是在血流中循环的小细胞，有助于凝血。血小板减少症 (thrombocytopenia) 是指血液内的血小板太少。 免疫性血小板减少症是指由于自身 免疫系统 免疫系统概述 免疫系统是您身体的防御系统。有助于保护您免受疾病和感染。免疫系统的工作是攻击不属于您体内的东西，包括： 微生物（如细菌、病毒和真菌） 寄生虫 癌细胞 其它可以进入体内的东西，如花粉 阅读更多 的破坏导致体内血小板太少。 血小板太少使你容易出血 你的皮肤上可能有小紫斑，并出现鼻出血和牙龈出血 医生根据血液检查的结果诊断 ITP 在儿童中，ITP 通常自行消失 ITP 成人患者可使用皮质类固醇或其他药物来降低免疫系统的活动度 如果你是成人并且药物治疗无效，医生可能会切除你的脾脏 是什么原因导致了 ITP？ 在 免疫系统 免疫系统概述 免疫系统是您身体的防御系统。有助于保护您免受疾病和感染。免疫系统的工作是攻击不属于您体内的东西，包括： 微生物（如细菌、病毒和真菌） 寄生虫 癌细胞 其它可以进入体内的东西，如花粉 阅读更多 生成 抗体 免疫系统的主要部分是什么？ 攻击和破坏血小板时，会发生 ITP。医生不知道为什么会发生这种情况，但在儿童中，它常常发生在病毒感染之后。 ITP 的症状有哪些？ ITP 的症状可能突然发生或缓慢发展。你可能出现： 皮肤上或口腔内有小红斑 发生极轻微损伤后也会淤青 牙龈出血 月经期大量出血 血小板计数下降导致你更容易出血。血小板数目极少的患者可能会出现严重的肠道出血，或危及生命的脑内出血。 医生如何判断我是否患有 ITP？ 医师会做： 做血液检查，以计算血液样本中的血小板数目 做检查明确是否存在引起血小板计数低的其他原因 罕见情况下，医生会采集一小块骨髓样本（骨髓活检），明确身体生成血小板的功能是否正常。 医生如何治疗 ITP？ 医生采用以下方法治疗 ITP： 皮质类固醇 帮助身体生成更多血小板的药物 降低免疫系统活动度的药物 极少数情况下，手术切除脾脏（脾切除术） 如果你出现危及生命的出血，医生可能会给你输注血小板。血小板输注通常效果不佳，因为血液中的抗体也会攻击输注的血小板。 切除脾脏有助于将血小板更多地保留在血液循环中。 在成人中，ITP 通常长时间持续存在，但儿童 ITP 一般会自行好转。

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Platelet Disorders - Immune Thrombocytopenia (ITP) | NHLBI, NIH

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HomeHealth Topics < Back To Platelet DisordersImmune Thrombocytopenia (ITP)

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Platelet Disorders Immune Thrombocytopenia (ITP)

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What is ITP?

Immune thrombocytopenia (ITP) is a type of platelet disorder. In ITP, your blood does not clot as it should, because you have a low platelet count. Platelets are tiny blood cells that are made in the bone marrow. When you are injured, platelets stick together to form a plug that seals your wound. This plug is called a blood clot. When you have a low platelet count, you may have trouble stopping bleeding.

ITP can be acute (short-term) or chronic (long-term). Acute ITP often lasts less than 6 months. It mainly occurs in children — both boys and girls — and is the most common type of ITP. Chronic ITP lasts 6 months or longer and mostly affects adults. However, some teens and children do get this type of ITP. Chronic ITP affects women two to three times more often than it affects men.

What are the symptoms?

ITP may not cause any symptoms. However, ITP can cause bleeding that is hard to stop. This bleeding can be inside your body, underneath your skin, or from your skin.

Signs of bleeding may include:

Petechiae, which are small, flat red spots under the skin caused by blood leaking from blood vessels

Purpura, which is bleeding in your skin that can cause red, purple, or brownish- yellow spots

Clotted or partially clotted blood under your skin (called a hematoma) that looks or feels like a lump

Nosebleeds or bleeding from your gums

Blood in your urine or stool

Heavy menstrual bleeding

Extreme tiredness

Purpura and petechiae in the skin. The photograph shows two types of bruising that are common in people who have ITP. The larger red, brown, and purple dots are purpura, and the smaller red and purple dots are petechiae.

How is it diagnosed?

To diagnose ITP, your provider will ask about your medical and family history. They will also ask about your symptoms and do a physical exam to look for signs of bleeding.

Your provider may order one or more of the following blood tests.

Complete blood count (CBC): This test measures your platelet count and the number of other blood cells in your blood.

Blood smear: For this test, some of your blood is put on a slide. A microscope is used to look at your platelets.

Bone marrow tests: These tests check whether your bone marrow is healthy. You may need this test to confirm that you have ITP and not another platelet disorder, especially if your treatment is not working.

You also may have a blood test to check for the

antibodies

that attack platelets.

If you’re at risk for HIV, hepatitis C, or Heliobacter pylori, your provider may screen you for these infections, which might be linked to ITP.

What causes ITP?

ITP is caused by problems with your

immune system

. Normally, your immune system helps your body fight off infections and diseases. In ITP, however, your immune system attacks and destroys your body’s platelets by mistake. You may also make fewer platelets. Why this happens is not known.

Some things that can raise your risk of ITP include:  

Antibiotics, antiviral medicines, or medicines to treat inflammation

Viral or bacterial infections, which can trigger your immune system to start destroying your platelets

Vaccines, such as the measles-mumps-rubella (MMR) vaccine, which, rarely, can raise the risk of ITP, especially in children

How is ITP treated?

For most children and adults, ITP is not a serious condition. Acute ITP in children often goes away on its own within a few weeks or months and does not return. For a small number of children, ITP does not go away on its own, and the child may need treatment.

Chronic ITP varies from person to person and can last for many years. Even people who have serious types of chronic ITP can live for decades. Most people who have chronic ITP can stop treatment at some point and maintain a safe platelet count.

Treatment depends on your platelet count and whether you have any symptoms. In mild cases, you may not need any treatment, and your provider will monitor your condition to make sure that your platelet count does not become too low. If you need treatment, your treatment plan may include medicines and procedures. If your ITP was caused by an infection, treating the infection may help increase your platelet count and lower your risk of bleeding problems.

Medicines

Medicines often are used as the first treatment for both children and adults.

Corticosteroids

, such as prednisone and dexamethasone, are commonly used to treat ITP. These medicines help increase your platelet count. However, steroids have many side effects. Some people relapse (get worse) when treatment ends. 

Other medicines used to raise the platelet count include:  

Eltrombopag

Immune globulin

Rituximab

Romiplostim

Removal of your spleen (splenectomy)

Doctors can surgically remove your spleen if necessary. The spleen is an organ in your upper left abdomen. It makes antibodies that help fight infections. In ITP, these antibodies destroy platelets by mistake.

Removing your spleen may raise your risk of infections. Before you have the surgery, your doctor may give you vaccines to help prevent infections. They will explain what steps you can take to help avoid infections and what symptoms to watch for.

Platelet transfusions

Some people who have ITP with serious bleeding may need to have a platelet transfusion. This is done in a hospital. Some people will need platelet transfusions before having surgery.

For a platelet transfusion, donor platelets from a blood bank are injected into your bloodstream. This increases your platelet count for a short time. Learn more about platelet transfusions.

What health problems can ITP cause?

Without proper treatment, ITP can cause serious bleeding and pregnancy complications. Learn how to manage ITP to help avoid complications.

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ITP-Immune Thrombocytopenic Purpura - StatPearls - NCBI Bookshelf

ITP-Immune Thrombocytopenic Purpura - StatPearls - NCBI Bookshelf

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NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. StatPearls [Internet].Show detailsTreasure Island (FL): StatPearls Publishing; 2024 Jan-.Search term

ITP-Immune Thrombocytopenic PurpuraAngel A. Justiz Vaillant; Nagendra Gupta.Author Information and AffiliationsAuthorsAngel A. Justiz Vaillant1; Nagendra Gupta2.Affiliations1 University of the West Indies2 Texas Health ResourcesLast Update: May 6, 2023.Continuing Education ActivityImmune thrombocytopenic purpura (ITP) is an autoimmune pathology characterized by a low platelet count, purpura, and hemorrhagic episodes caused by antiplatelet autoantibodies. The exclusion typically makes the diagnosis of the known causes of thrombocytopenia. IgG autoantibodies sensitize the circulating platelets. It leads to the accelerated removal of these cells by antigen-presenting cells (macrophages) of the spleen and sometimes the liver or other components of the monocyte-macrophage system. Bone marrow compensates the platelet destruction by increasing platelet production. ITP most often occurs in healthy children and young adults within a few weeks following a viral infection. Certain drugs can also cause immune thrombocytopenia indistinguishable from ITP. Most children have spontaneous remission within a few weeks or months, and splenectomy is rarely needed. However, young adults rarely have spontaneous remissions necessitating splenectomy within the first few months after diagnosis. According to current evidence, this activity will review the most common causes of ITP and outline an interprofessional treatment approach.

Objectives:

Describe the pathophysiology of immune thrombocytopenic purpura (ITP).Review the causes of immune thrombocytopenic purpura (ITP).Summarize the treatment options for immune thrombocytopenic purpura (ITP).Explain how the facilitation of interprofessional team education and discussion can optimize the effective detection of ITP and inform the need for subsequent evaluations.

Access free multiple choice questions on this topic.

IntroductionImmune thrombocytopenic purpura (ITP) is an autoimmune disease characterized by a low platelet count, purpura, and hemorrhagic episodes caused by antiplatelet autoantibodies. The diagnosis is typically made by excluding the known causes of thrombocytopenia. IgG autoantibodies sensitize the circulating platelets. It leads to the accelerated removal of these cells by antigen-presenting cells (macrophages) of the spleen and sometimes the liver or other components of the monocyte-macrophage system. The bone marrow compensates for platelet destruction by increasing platelet production. ITP most often occurs in healthy children and young adults within a few weeks following a viral infection.ITP is usually manageable with immunosuppressive therapy.[1][2] An identical form of autoimmune thrombocytopenia can also be associated with chronic lymphocytic leukemia, lymphomas, SLE, infectious mononucleosis, and other bacterial and viral infections. Certain drugs can also cause immune thrombocytopenia indistinguishable from ITP. Most children have spontaneous remission within a few weeks or months, and splenectomy is rarely needed. However, young adults rarely have spontaneous remissions necessitating splenectomy within the first few months after diagnosis. The International Working Group on ITP defines ITP according to the following clinical phases.[3] These are as follows:Newly diagnosed ITP is in the first three months post-diagnosis.Persistent ITP is for 3-12 months.Chronic ITP is for > 12 months.Refractory ITP is the failure of splenectomy.EtiologyImmune thrombocytopenic purpura can occur with infections (e.g., human immunodeficiency virus), malignancy (e.g., adenocarcinoma and lymphoma), and common variable immunodeficiency and autoimmune diseases (e.g., systemic lupus erythematosus, autoimmune hepatitis, and thyroid disease).[4] In these diseases, anti-platelet antibodies form, leading to platelet destruction. Drugs such as acetazolamide, aspirin, aminosalicylic acid, carbamazepine, cephalothin, digitoxin, phenytoin, meprobamate, methyldopa, quinidine, rifampin, and sulfamethazine may also cause autoimmune thrombocytopenia,  Autoantibody production against platelets is advocated as one etiology for ITP.[5] The modern theory also considers the possibility of a failure of the self-tolerance mechanism. EpidemiologyImmune thrombocytopenic purpura can be divided into two classifications; acute and chronic. The acute form presents in childhood, affects both sexes, and may be prefaced by a viral infection. Most children (85%) have a benign course and do not require treatment. They can spontaneously recover within three months. The chronic form affects individuals between ages 20 to 50 years; there is a female/male ratio of 3 to 1, and It is usually not preceded by a viral infection. The female preponderance is thought to have some relationship to the increased prevalence of autoimmune disease in women.[3] It may present with bleeding episodes for months or years; during that time, the platelet counts are close to normal. Fewer than 10% of children develop chronic ITP.PathophysiologyThe spleen is an important site of autoantibody production. Sequestration of anti-platelet IgG antibodies occurs in the spleen's red pulp, where sensitized platelet removal occurs by phagocytosis. Research showed that radiolabeled-IgG sensitized platelet removal occurs in a few hours compared with a normal platelet half-life of 8 to 9 days.[2]In contrast to maternal ITP, gestational thrombocytopenia rarely brings the count below 70,000 /dL, typically does not cause bleeding, and has its origins in a dilutional, not consumptive, mechanism.[3]Neonatal alloimmune thrombocytopenia may occur in pregnant women who are negative for the platelet antigen PL a1 but were sensitized in prior pregnancies by infants who were PL a1 positive or by blood transfusion. The condition has also involved other platelet antigens.[6] Pregnant women with ITP have an increased incidence of fetal loss, a low fetal birth rate, and a higher incidence of premature births.[3]In drug-induced ITP, the drug absorbs the platelet cell membrane. The immune system makes antibodies to the target drug-platelet complex, which results in the removal of the sensitized platelet by phagocytes residing in the spleen and liver. The activation of the complement system by the classical pathway is another effector mechanism of platelet cell damage (thrombocytopenia).[7] Childhood immune thrombocytopenic purpura often occurs within a few weeks following a viral infection, suggesting a possible cross-immunization between viral and platelet antigens, the absorption of immune complexes, or a hapten mechanism.Many other platelet antigens are a target of autoantibodies, including GPIIb/IIIA and GP V (after chickenpox). However, their exact role in diagnostic testing is dubious at best.[3]HistopathologyHistopathology of immune thrombocytopenic purpura can often reveal the increased production of megakaryocytes in the bone marrow.[8] This finding suggests that thrombocytopenia is secondary to increased platelet destruction rather than decreased platelet formation. Harrington and coworkers first showed in 1951 that the plasma from a patient with immune thrombocytopenic purpura caused thrombocytopenia when transfused into a healthy subject.History and PhysicalThere may be a history of drug use, a viral infection, or immunization. Acute immune thrombocytopenic purpura can be characterized by generalized purpura in a previously healthy child or, less commonly in an adult, bruises following minor trauma, the presence of oral hemorrhagic bullae, epistaxis, gastrointestinal bleeding, conjunctival hemorrhage, and hematuria. More commonly, the illness is gradual in onset but chronic. Severe bleeding has been noted in as many as 9.5% of adults.[9]Chronic immune thrombocytopenic purpura may be characterized by insidious onset or suddenly becomes acute. It is seen more commonly in females and presents with scattered petechiae, epistaxis, and menorrhagia, episodes of bleeding separated by an extended period. Occasionally, these clinical findings can be due to HIV-related illness.EvaluationThe laboratory tests will show the following:Low platelet count, usually <40x10^9/L for over three months.Blood film: It shows large platelets and tiny platelet fragments.Bone marrow examination: It shows an increased number of megakaryocytes.Platelet Coomb's test: Detects anti-platelet antibodies fixed on the patient's platelets. However, it should also be noted that antibody analysis focal to the platelet glycoproteins IIb/IIIa, Ib/IX, and Ia/IIa are of low sensitivity and seldom efficacious.[3]Indirect test: Uses a pool of normal donor platelets to detect free serum antibodies against platelets, usually anti-glycoprotein IIb/IIIa antibodies. Various other tests can detect anti-platelet antibodies, including lymphocyte activation by autologous platelets, lymphocyte activation by platelet-antibody immune complexes, phagocytosis of platelet-associated IgG by competitive binding assays, radiolabeled Coombs antiglobulin test, fluorescein-labeled Coombs antiglobulin, and ELISA. Testing for systemic lupus erythematosus (presenting with ITP):Antinuclear antibodies (ANA) can be performed using indirect immunofluorescence. Most cases of SLE show positive ANA results.  There is clinical data to suggest that ITP patients are at high risk of developing SLE [10].  It was noted in one study that 12.8% of patients with SLE were initially diagnosed with ITP.  The search for one should press consideration of the other.   Testing for auto-antibodies: This includes testing for anti-double-stranded DNA (anti-dsDNA), anti-Smith, ENA, anti-cardiolipin, and anti-beta2 GP-I antibodies. A high serum level of anti-dsDNA and anti-Smith antibodies suggests SLE.   Additionally, detection of C3 and C4, immunoglobulins (IgM, IgG, and IgA), serum protein electrophoresis, and cryoglobulins (if Raynaud is present) may be performed.Biopsies (lupus band test): Shows IgG and C3/C4 deposits along the dermo-epidermal junction in a lumpy-bumpy distribution. A renal biopsy may be helpful. It shows the deposition of immunocomplexes in the glomeruli. Testing for HIV and Hepatitis C is advocated as these are treatable causes of thrombocytopenia.[3]Treatment / ManagementThe management of ITP involves several goals and concepts.[11][1][12] These include the following:Aim to bring the platelet count to normal.  Indication for active therapy is only when there is acute bleeding or if emergent surgery is forthcoming. If there is no significant bleeding and the platelet count is > 30,000 /dL, then transfusion may be withheld.[3]  In a recent analysis, severe bleeding was seen in about 20% of children and about 10% of adults. Bleeding usually does not occur until the platelet count decreases to below 30,000 /dL. Cutaneous and mucosal bleeding may occur below 20,000 /dL, and life-threatening hemorrhage (e.g., intracranial) typically occurs with counts below 10,000 /dL. It is preferred to give one apheresis unit; 4 to 6 pooled platelet units may also be given. The vast majority of children recover spontaneously without sequelae. In severe cases, the treatment of choice is intravenous immunoglobulin (IV IgG). A 5-day course of 400 mg/kg/d is given. Responses occur in more than 70% in 1 to 4 days, but only for a short period; many patients respond, and repeated courses may be necessary.   Patients (children and adults) with active bleeding require corticosteroids to stop further destruction of platelets (about 60% of patients respond well within two weeks). Steroid use in pregnant women during the first trimester carries a small risk of cleft palate.[13] Dexamethasone use in pregnancy can cause an increased risk of abruptio placenta as well as premature rupture of fetal membranes. Patients who do not respond adequately and have active bleeding after a month of being treated with corticosteroids may need splenectomy after using intravenous immunoglobulins to raise the platelet count. Therefore, splenectomy is the treatment of choice for adult patients with ITP who have persistent symptomatic thrombocytopenia. It is recommended that patients be screened for COVID prior to splenectomy.[13] Splenectomy results in upwards of a 70% long-term response rate; it is important to give vaccinations > 15 days prior to surgery. In adult patients with ITP, the corticosteroid preferred is prednisone 1 to 2 mg/kg/d. The response rate for a seven-day course is about 56%, with a long overall remission rate.[9] Steroid administration beyond a 6-week course is not advocated.[13] It reserves plasmapheresis for cases of fulminant ITP.[14]Neonatal thrombocytopenia is treatable with intravenous immunoglobulin, which raises the platelet count.  IV-IgG is favored over Anti-D immune globulin due to the increased risk of severe hemolytic transfusion reactions in the latter.[3]Platelets increase after three months in untreated ITP neonates due to the catabolism of maternal anti-platelet antibodies transferred during birth.  Vincristine (VCR) seems to be a valuable drug in adult patients that do not respond to splenectomy. However, VCR can cause neuropathy, and it is contraindicated in pregnancy.[9]Hemostaticxs such as tranexamic acid and Epsilon Aminocaproic Acid are not favored in treating ITP.[3]Rituximab use in ITP appears limited to second-line deployment, likely as combination therapy.[13] While it carries a > 50% response rate, its use is fraught with potential difficulties. A hepatitis panel must be checked prior to use in case of possible Hepatitis B reactivation. There is a small risk of progressive Multifocal Leukoencephalopathy (PML). With rituximab administration, COVID vaccinations cannot be given for 6 to 12 months. There is also a possible (20%) risk of hypogammaglobulinemia (requiring IV-IgG replacement) if rituximab is coadministered with dexamethasone. Rituximab can also cross the placenta leading to neonatal B-cell depletion. Anti-RhD globulin is immunoglobulin directed against the D-antigen of the Rh blood group.[15] It inhibits the destruction of antibody-coated platelets. Its utility is limited to Rh+ patients with a spleen. The response rate is approximately 70%, with almost all being complete responses (CR) and 26% of these had sustained responses beyond six months.[16] The use of Anti-RhD in pregnant women is tempered by the increased risk of neonatal jaundice and anemia.[13]Fostamatinib is a splenic tyrosine kinase inhibitor that decreases antibody-dependent phagocytosis of platelets.[5][17] The overall response rate was 43% within 12 weeks of therapy, and 18% have stable disease.[13][18] Responses can occur despite prior failures with thrombopoietins, rituximab, or splenectomy. Efgartigimod and rozanolixizumab are compounds undergoing testing for use in myasthenia gravis as well as in ITP.[5][19] They are antibody fragments that target the neonatal Fc-receptor (FcRn), thereby inhibiting IgG recycling. They decrease the half-life of IgG, reducing it to normal or subpathogenic levels. Investigators have found response rates of 38% with efgaritigimod and 50% with Rozanolixizumab. Sutimlimab is a humanized monoclonal antibody against C1s, a complement pathway inhibitor.[20] In ITP, it decreases complement-dependent cytotoxicity, thereby decreasing platelet destruction.[5] Studies have revealed responses of 33%, many within days. However, their overall sample sizes were small. Rilzabrutinib is a Bruton Tyrosine Kinase-inhibitor (BTKI) with efficacy in the treatment of ITP.[5][18] It inhibits Fc (gamma) signal transduction causing a decrease in platelet phagocytosis and auto-antibody production. Studies have shown a response rate of about 40 to 50%, with minimal toxicity and a time to respond of about a week. Azathioprine may take several months of therapy to exert an effect on ITP.[9] Although deemed safe in pregnancy, there are reports of an increased rate of prematurity as well as a decreased birth rate.[13] In general, vaccinations must be withheld for six months after the total cessation of immunosuppressive therapy to allow for the restoration of immunity. Thrombopoietins (eltrombopag, romiplostim, and avatrombopag) have been shown in ITP to have upwards of an 80% response rate.[13] These medicines stimulate the JAK-STAT pathway with subsequent megakaryocytic proliferation and platelet production.[5] There is an inherent risk of thrombosis, which increases substantially if the patient is on oral contraceptives, has an underlying antiphospholipid antibody, or has other prothrombotic issues. Problems typically occur within a year of use. In recent clinical trials in children with ITP, treated with Romiplostin, that about half demonstrated a positive platelet response within the first 6 months [21]   Bone marrow biopsies obtained during therapy revealed that reticulin deposition had no correlative effect with the positive platelet response.  Clinically significant fibrosis occurred rarely with prolonged therapy.  The Korean Society of Hematology recommended using thrombopoietins (TPO) after steroid failure but before considering splenectomy.[9] It was felt that the shortcomings of surgery outweighed those of this medical management.  Recent opinion appears to support the use of combined agents in the therapeutic approach to ITP.[18][22][23] A common format involves giving steroids along with IV-IgG and adding platelets should the patient be bleeding.[3] The combination of mycophenolate mofetil or cyclosporine with thrombopoietin and IV-IgG has been seen to have a response rate of 72% in severe refractory ITP. Studies combining Dexamethasone, Cyclosporine, and low-dose rituximab revealed a response rate of 60%, often with a long-term (> 7 months) remission. Rituximab with thrombopoietin has fared better than rituximab alone. The overall response rate ()RR) was about 79% for the combination compared to about 71% for monotherapy. The combination did carry a shorter response time but no long-term advantage. Differential DiagnosisOne should consider the following in the differential thrombocytopenias due to increased platelet destruction and those due to decreased platelet production.[1][24][25]

Thrombocytopenias due to increased platelet destruction include:

Immune thrombocytopeniaIdiopathic thrombocytopenic purpuraSecondary autoimmune thrombocytopeniasDrug-induced immune thrombocytopeniasPosttransfusion purpura (PTP) is a transfusion-associated reaction occurring about a week after the transfusion. It is an amnestic response to platelet antigens resulting in the destruction of native and transfused platelets.[26] It occurs in multiparous women due to alloimmunization against platelet antigens.[27] It is treated with IV-IgG. Neonatal immune thrombocytopeniasViral infection often is the presage for thrombocytopenia. The HIV-associated disease is now the most common cause of thrombocytopenic purpura, especially in males between 20 and 50. Testing for HIV antibodies is a critical part of the assessment of ITP.[28][29] Zidovudine effectively raises platelet counts in individuals with HIV-associated immune thrombocytopenic purpura. Data has also shown that COVID is a cause of thrombocytopenia, with 71% of COVID-induced ITP occurring in elderly patients and three-quarters of these having moderate to severe infections.[3] Typically the platelet count does not drop below 100,000 /dL, severe bleeding is uncommon, and the overall prognosis is deemed relatively favorable. Incidentally, COVID vaccines have been implicated in causing and exacerbating ITP [30].  Hepatitis C can cause significant thrombocytopenia, which can resolve with antiviral treatment.[31][32] Consumptive thrombocytopeniasThrombotic thrombocytopenic purpura (TTP); Of special note is that TTP and ITP, though thought of as distinct hematopoietic maladies both subset autoimmune disorders [30].  Clinical examples exist of concurrent or sequential presentations.  A phenotypic distinction can sometimes be difficult but a necessity in terms of selecting the proper treatment (eg. plasm exchange, steroids, etc.). Disseminated intravascular coagulation (DIC) Hemolytic-uremic syndrome (HUS)Microangiopathic hemolytic anemias such as TTP, DIC, and HUS can manifest thrombocytopenias as blood flow is disrupted and erythrocytes are destroyed as platelets are consumed.[33][34]Sepsis causes thrombocytopenia via various mechanisms, such as complement activation, histone release, and coagulation activation.[35]Hypersplenism; sequestration mechanism. Thrombocytopenias, due to decreased platelet production, colloquially described as "the first to go and the last to grow back," platelet production and survival are affected by a myriad of factors, sometimes with long-term results.[35]  Bone marrow suppression by drugs, alcohol, toxins, and infections Aplastic anemia; manifests as pancytopenia, typically from a damaged marrow manifesting marrow failure.[36]Leukemias and other bone marrow cancers; the patient may present with thrombocytopenia within a myelophthisic picture or with leukoerythroblastocic.[37]  Megaloblastic anemia; thrombocytopenia can appear in B12 deficiency, especially as this disease can mimic TTP.[38]  Refractory anemias, preleukemia, and hematopoietic dysplasia; typically has cellular dysmorphism evident on bone marrow exam and often presents as bilineage cytopenias.[39]PrognosisThe prognosis is good for acute ITP since most patients make a spontaneous recovery. Patients with chronic ITP almost always require treatment, and relapse commonly occurs. A complete response to the first-line steroid can occur in about 20% of the patients, and about 60% have a partial response. Vincristine is used in adult patients that do not respond to splenectomy.ComplicationsThe most severe complication of ITP is hemorrhage, especially the rare complication of bleeding into the brain that may prove fatal.Deterrence and Patient EducationThe general practitioner can assess the bleeding and make recommendations about the management, and also a referral to a specialist (hematologist). The lack of definitive testing for ITP makes the diagnostic approach one of the elimination of possible causes, ergo, a diagnosis of exclusion.[40] Explaining the treatment of a 'presumptive' diagnosis to a patient may be a difficult task, but one made better if the patient improves with the modalities described here. Patient compliance is a priority. Pearls and Other IssuesThe definition of thrombocytopenia is when the blood platelet count is < 150x10^9/L. It may not be symptomatic until the platelet count falls below 10x10^9/L.  ITP can be idiopathic, although it is often autoimmune-related.   ITP can be secondary to SLE, HIV, and drugs (e.g., quinine).   The antibody involved is not temperature-dependent and always directed against platelet-specific antigens.    Corticosteroids work by decreasing phagocytosis of antibody-coated platelet by phagocytes in both the spleen and liver. Splenectomy removes the sites of autoantibody production and phagocytosis and is successful in most patients.    In refractory patients, one can successfully use intravenous immunoglobulins. The IgG blocks Fc-receptors on macrophages and reduces platelet binding to autoantibodies.Enhancing Healthcare Team Outcomes An interprofessional team should manage the patient with ITP. The clinicians and nursing staff may monitor subjects with a less severe disease if it does not involve important organ systems. The clinician should refer patients with complications to a hematologist, coordinating closely with the patient's clinician to maximize management and progress. The pharmacist should be involved in the coordination of drug therapy and monitoring for complications and interactions. All interprofessional team members must maintain accurate records of all interactions and interventions with the patient so all personnel involved in care have access to the same updated patient information. Open lines of communication are essential to ensure proper and timely care, particularly if any concerns arise, such as adverse effects or a deterioration in the patient's condition. An interprofessional team approach will result in the best outcomes. [Level 5]Review Questions

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[PMC free article: PMC8958378] [PubMed: 35342042]10.Pamuk ON, Ali SM, Hasni S. Development of systemic lupus erythematosus in patients with immune thrombocytopenic purpura: A systematic meta-analysis. Autoimmun Rev. 2023 Apr;22(4):103297. [PMC free article: PMC10078978] [PubMed: 36781038]11.Apte S, Navarro-Puerto J, Damodar S, Ramanan V, John J, Kato G, Ross C, Shah C, Torres M, Fu C', Rucker K, Pinciaro P, Barrera G, Aragonés ME, Ayguasanosa J. Safety and efficacy of intravenous immunoglobulin (Flebogamma® 10% DIF) in patients with immune thrombocytopenic purpura. Immunotherapy. 2019 Feb;11(2):81-89. [PubMed: 30499734]12.George JN. Sequence of treatments for adults with primary immune thrombocytopenia. Am J Hematol. 2012 May;87 Suppl 1:S12-5. [PubMed: 22389032]13.Bussel JB, Garcia CA. Diagnosis of immune thrombocytopenia, including secondary forms, and selection of second-line treatment. Haematologica. 2022 Sep 01;107(9):2018-2036. [PMC free article: PMC9425307] [PubMed: 35708136]14.Branda RF, Tate DY, McCullough JJ, Jacob HS. Plasma exchange in the treatment of fulminant idiopathic (autoimmune) thrombocytopenic purpura. Lancet. 1978 Apr 01;1(8066):688-90. [PubMed: 76226]15.Kim DS. Recent advances in treatments of adult immune thrombocytopenia. Blood Res. 2022 Apr 30;57(S1):112-119. [PMC free article: PMC9057657] [PubMed: 35483935]16.Lakhwani S, López-Las Heras A, Rodríguez-García P, Iraheta S, Martín-Santos T, Rodríguez-Salazar MJ, Machado P, Hernández MT. Intramuscular Anti-D treatment for immune thrombocytopenia: A single centre experience. Br J Haematol. 2023 Feb;200(3):353-357. [PMC free article: PMC10091692] [PubMed: 36198407]17.Mehta AR, Kefela A, Toste C, Sweet D. Real-World Use of Fostamatinib in Patients with Immune Thrombocytopenia and Thrombotic Risk. Acta Haematol. 2022;145(2):221-228. [PMC free article: PMC9116595] [PubMed: 34913873]18.Lv Y, Shi H, Liu H, Zhou L. 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"Immune" Thrombocytopenia as Key Feature of a Novel ADA2 Deficiency Variant: Implication on Differential Diagnostics of ITP in Children. J Pediatr Hematol Oncol. 2019 Mar;41(2):155-157. [PubMed: 29620681]26.Hawkins J, Aster RH, Curtis BR. Post-Transfusion Purpura: Current Perspectives. J Blood Med. 2019;10:405-415. [PMC free article: PMC6910090] [PubMed: 31849555]27.Rafei H, Yunus R, Nassereddine S. Post-Transfusion Purpura: A Case Report of an Underdiagnosed Phenomenon. Cureus. 2017 May 01;9(5):e1207. [PMC free article: PMC5451204] [PubMed: 28580204]28.Ponzetto A, Figura N, Fiorini G. Immune thrombocytopenic purpura and infections. Intern Emerg Med. 2018 Oct;13(7):1137. [PubMed: 30030700]29.Okazaki M, Nakamura M, Imai A, Asagiri T, Takeuchi S. Sequential occurrence of Graves' disease and immune thrombocytopenic purpura as manifestations of immune reconstitution inflammatory syndrome in an HIV-infected patient. Int J STD AIDS. 2018 Jul;29(8):834-836. [PubMed: 29361886]30.Lin HC, Huang J, Huang J, Zhang LJ, Yin XW, Yang JC, Huang XY. Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura: a case report and review of the literature. J Med Case Rep. 2023 Feb 08;17(1):38. [PMC free article: PMC9905008] [PubMed: 36750960]31.Yoshida M, Tateishi R, Hiroi S, Fujiwara M, Kitanishi Y, Iwasaki K, Takeshima T, Igarashi A. Changes in Platelet Counts and Thrombocytopenia Risk in Patients with Chronic Liver Disease with Different Etiologies Using Real-World Japanese Data. Adv Ther. 2022 Feb;39(2):992-1003. [PMC free article: PMC8866341] [PubMed: 34928469]32.Tsai TL, Jhou HM, Fan FS. Conspicuous Response to Direct-Acting Antivirals in Chronic Hepatitis C-related Immune Thrombocytopenia: A Case Report. Cureus. 2022 Apr;14(4):e24193. [PMC free article: PMC9110079] [PubMed: 35592216]33.Kottke-Marchant K. Diagnostic approach to microangiopathic hemolytic disorders. Int J Lab Hematol. 2017 May;39 Suppl 1:69-75. [PubMed: 28447417]34.Arnold DM, Patriquin CJ, Nazy I. Thrombotic microangiopathies: a general approach to diagnosis and management. CMAJ. 2017 Jan 30;189(4):E153-E159. [PMC free article: PMC5266569] [PubMed: 27754896]35.Santoshi RK, Patel R, Patel NS, Bansro V, Chhabra G. A Comprehensive Review of Thrombocytopenia With a Spotlight on Intensive Care Patients. Cureus. 2022 Aug;14(8):e27718. [PMC free article: PMC9356658] [PubMed: 35949449]36.Young NS. Aplastic Anemia. N Engl J Med. 2018 Oct 25;379(17):1643-1656. [PMC free article: PMC6467577] [PubMed: 30354958]37.Mahdi EJ, Mahdi AJ. Leucoerythroblastosis and thrombocytopenia as clues to metastatic malignancy. BMJ Case Rep. 2014 Jan 31;2014 [PMC free article: PMC3912367] [PubMed: 24488663]38.Torrez M, Chabot-Richards D, Babu D, Lockhart E, Foucar K. How I investigate acquired megaloblastic anemia. Int J Lab Hematol. 2022 Apr;44(2):236-247. [PubMed: 34981651]39.Dotson JL, Lebowicz Y. StatPearls [Internet]. StatPearls Publishing; Treasure Island (FL): Jul 18, 2022. Myelodysplastic Syndrome. [PubMed: 30480932]40.Visweshwar N, Ayala I, Jaglal M, Killeen R, Sokol L, Laber DA, Manoharan A. Primary immune thrombocytopenia: a 'diagnosis of exclusion'? Blood Coagul Fibrinolysis. 2022 Sep 01;33(6):289-294. [PMC free article: PMC9415225] [PubMed: 35867940]Disclosure: Angel Justiz Vaillant declares no relevant financial relationships with ineligible companies.Disclosure: Nagendra Gupta declares no relevant financial relationships with ineligible companies.

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ViewsPubReaderPrint ViewCite this PageJustiz Vaillant AA, Gupta N. ITP-Immune Thrombocytopenic Purpura. [Updated 2023 May 6]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. In this PageContinuing Education ActivityIntroductionEtiologyEpidemiologyPathophysiologyHistopathologyHistory and PhysicalEvaluationTreatment / ManagementDifferential DiagnosisPrognosisComplicationsDeterrence and Patient EducationPearls and Other IssuesEnhancing Healthcare Team Outcomes Review QuestionsReferencesBulk DownloadBulk download StatPearls data from FTPRelated informationPMCPubMed Central citationsPubMedLinks to PubMedSimilar articles in PubMedReview [Idiopathic thrombocytopenic purpura in children].[Med Pregl. 1998]Review [Idiopathic thrombocytopenic purpura in children].Gebauer E, Vijatov G. Med Pregl. 1998 Mar-Apr; 51(3-4):127-34. Review The pathogenesis of chronic immune thrombocytopenic purpura.[Semin Hematol. 2007]Review The pathogenesis of chronic immune thrombocytopenic purpura.McMillan R. Semin Hematol. 2007 Oct; 44(4 Suppl 5):S3-S11. Review [Diagnostic approach and treatment of immune thrombocytopenia in adults].[Acta Med Croatica. 2013]Review [Diagnostic approach and treatment of immune thrombocytopenia in adults].Kolonić SO, Patekar MB, Milunović V. Acta Med Croatica. 2013 Mar; 67(1):3-11. Review Review: immune thrombocytopenic purpura: an update for immunohematologists.[Immunohematology. 2004]Review Review: immune thrombocytopenic purpura: an update for immunohematologists.Sandler SG. Immunohematology. 2004; 20(2):112-7. Review Refractory immune thrombocytopenic purpura (ITP) after accessory splenectomy: A case report and literature review.[Clin Hemorheol Microcirc. 2023]Review Refractory immune thrombocytopenic purpura (ITP) after accessory splenectomy: A case report and literature review.Riaz A, Ali HT, Ali F, Ali J. Clin Hemorheol Microcirc. 2023; 85(2):189-194. See reviews...See all...Recent ActivityClearTurn OffTurn OnITP-Immune Thrombocytopenic Purpura - StatPearlsITP-Immune Thrombocytopenic Purpura - StatPearlsYour browsing activity is empty.Activity recording is turned off.Turn recording back onSee more...

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Idiopathic Thrombocytopenic Purpura | Johns Hopkins Medicine

Idiopathic Thrombocytopenic Purpura | Johns Hopkins Medicine

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Idiopathic Thrombocytopenic Purpura

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What is immune thrombocytopenic purpura?Immune thrombocytopenic purpura (ITP) is a blood disorder characterized by a decrease in the number of platelets in the blood. Platelets are cells in the blood that help stop bleeding. A decrease in platelets can cause easy bruising, bleeding gums, and internal bleeding. This disease is caused by an immune reaction against one's own platelets. It has also been called autoimmune thrombocytopenic purpura.Thrombocytopenia means a decreased number of platelets in the blood.Purpura refers to the purple discoloring of the skin, as with a bruise.ITP is a fairly common blood disorder that both children and adults can develop.There are two forms of ITP:Acute thrombocytopenic purpura. This usually affects young children, ages 2 to 6 years old. The symptoms may follow a viral illness, such as chickenpox. Acute ITP usually starts suddenly and the symptoms usually disappear in less than 6 months, often within a few weeks. Treatment is often not needed. The disorder usually does not recur. Acute ITP is the most common form of the disorder.Chronic thrombocytopenic purpura. The onset of the disorder can happen at any age, and the symptoms can last a minimum of 6 months, several years, or a lifetime. Adults have this form more often than children do, but it does affect adolescents. Females have it more often than males. Chronic ITP can recur often and requires continual follow-up care with a blood specialist (hematologist).What causes idiopathic thrombocytopenic purpura?In ITP, the immune system is stimulated to attack your body's own platelets. Most often this is a result of antibody production against platelets. In a small number of cases, a type of white blood cell called T-cells will directly attack platelets. This immune system error may be a result of any of the following:Medications (including over-the-counter medications) can cause an allergy that cross-reacts with platelets.Infections, typically viral infections, including the viruses that cause chicken pox, hepatitis C, and AIDS, can prompt antibodies that cross-react with platelets.PregnancyImmune disorders, such as rheumatoid arthritis and lupusLow-grade lymphomas and leukemias may produce abnormal antibodies against platelet proteins.Sometimes the cause of immune thrombocytopenic purpura is not known.What are the symptoms of idiopathic thrombocytopenic purpura?Normal platelet count is in the range of 150,000 to 450,000. With ITP, the platelet count is less than 100,000. By the time significant bleeding occurs, you may have a platelet count of less than 10,000. The lower the platelet count, the greater the risk of bleeding.Because platelets help stop bleeding, the symptoms of ITP are related to increased bleeding. However, each person may experience symptoms differently. Symptoms may include:The purple color of the skin after blood has "leaked" under it. A bruise is blood under the skin. Persons with ITP may have large bruises from no known injury. Bruises can appear at the joints of elbows and knees just from movement.Tiny red dots under the skin that are a result of very small bleeds.NosebleedsBleeding in the mouth and/or in and around the gumsHeavy menstrual periodsBlood in the vomit, urine, or stoolBleeding in the head. This is the most dangerous symptom of ITP. Any head injury that occurs when there are not enough platelets to stop the bleeding can be life threatening.The symptoms of ITP may look like other medical problems. Always consult your health care provider for a diagnosis.How is idiopathic thrombocytopenic purpura diagnosed?In addition to a complete medical history and physical exam, you may have these tests:Complete blood count (CBC). A measurement of size, number, and maturity of different blood cells in a specific volume of blood (to measure platelets.Additional blood and urine tests. These tests are done to measure bleeding time and detect possible infections, including a special blood test called an antiplatelet antibody test.Careful review of your medicationsHistorically, a bone marrow aspiration was required to make a diagnosis of ITP. It may not be absolutely necessary in the face of a positive antiplatelet antibody test, but it is still commonly done to look at the production of platelets and to rule out any abnormal cells the marrow may be producing that could lower platelet counts. A bone marrow aspiration is necessary for a diagnosis if the antiplatelet antibody testing is negative.How is idiopathic thrombocytopenic purpura treated?Specific treatment for idiopathic thrombocytopenic purpura will be determined by your health care provider based on:Your age, overall health, and medical historyExtent of the diseaseYour tolerance for specific medications, procedures, or therapiesExpectations for the course of the diseaseYour opinion or preferenceWhen treatment is necessary, the two most common forms of immediate treatment are steroids and intravenous gamma globulin:Steroids. Steroids help prevent bleeding by reducing the rate of platelet destruction. Steroids, if effective, will result in an increase in platelet counts seen within 2 to 3 weeks. Side effects may include irritability, stomach irritation, weight gain, high blood pressure, and acne.Intravenous gamma globulin (IVGG). Intravenous gamma globulin (IVGG) is a protein that contains many antibodies and also slows the destruction of platelets. IVGG works faster than steroids (within 24 to 48 hours).Other treatments for ITP may include:Rh immune globulin. This medication temporarily stops the spleen from destroying platelets. You must be Rh positive and have a spleen for this medication to be effective.Medication changes. If it is a medication that is the suspected cause, discontinuation or changing the medication may be necessary.Infection treatment. If infection is the cause for ITP, then treatment of the infection may result in higher platelet counts.Splenectomy. In some cases, your spleen may need to be removed since this is the most active site of antibody mediated platelet destruction. This is considered more often in people with chronic ITP to decrease the rate of platelet destruction.Platelet transfusion. People with severe bleeding or about to go into surgery may require platelet transfusion.Rituximab (Rituxan). This medication is an antibody produced in a lab against a protein found on the blood cells that make antibodies. It slows the antiplatelet antibody production.Romiplostim (N-plate) and eltrombopag (Promacta). These medications were recently approved for the treatment of ITP that has failed other types of treatment. They stimulate the bone marrow to produce more platelets.Lifestyle changes. These can include making sure that you use protective gear and that you avoid certain activities.Key points about immune thrombocytopenic purpuraIdiopathic thrombocytopenic purpura is a blood disorder characterized by an abnormal decrease in the number of platelets in the blood.A decrease in platelets can result in easy bruising, bleeding gums, and internal bleeding.ITP may be acute and resolve in less than 6 months, or chronic and last longer than 6 months.Treatment options include a variety of medications that can reduce the destruction of platelets or increase their production.In some cases, surgery to remove the spleen is necessary.Next stepsTips to help you get the most from a visit to your health care provider:Before your visit, write down questions you want answered.Bring someone with you to help you ask questions and remember what your provider tells you.At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you.If you have a follow-up appointment, write down the date, time, and purpose for that visit.Know how you can contact your provider if you have questions.

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