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特发性血小板减少性紫癜:儿童及成人病理生理学与诊断的简明概述

Idiopathic thrombocytopenic purpura: A concise summary of the pathophysiology and diagnosis in children and adults.

作者信息

George J N, Raskob G E

机构信息

University of Oklahoma Health Sciences Center, Oklahoma City 73190, USA.

出版信息

Semin Hematol. 1998 Jan;35(1 Suppl 1):5-8.

PMID:9523743
Abstract

Idiopathic thrombocytopenic purpura (ITP) is a disorder that has distinct clinical manifestations in children and adults. In children, ITP is typically abrupt in onset and self-limited in its course. Girls and boys are equally affected. In adults, ITP is typically more indolent in its onset and the course is persistent, often lasting many years or characterized by recurrent exacerbations of disease. Most patients are young women. Intracerebral hemorrhage, while rare, is the most common cause of death in children and adults with ITP. In both children and adults, the ITP is assumed to be caused by antibody-mediated platelet consumption, where Fc receptors on the macrophage bind to antibody-coated platelets resulting in an accelerated platelet destruction. However, tests for antiplatelet antibodies have not yet been established as clinically useful for management decisions. The natural history and long-term prognosis of adults with ITP remain incompletely defined.

摘要

特发性血小板减少性紫癜(ITP)是一种在儿童和成人中具有不同临床表现的疾病。在儿童中,ITP通常起病急骤,病程呈自限性。男孩和女孩受影响的程度相同。在成人中,ITP通常起病较为隐匿,病程持续,常持续多年或表现为疾病的反复加重。大多数患者为年轻女性。脑出血虽然罕见,但却是儿童和成人ITP最常见的死亡原因。在儿童和成人中,ITP均被认为是由抗体介导的血小板消耗所致,巨噬细胞上的Fc受体与抗体包被的血小板结合,导致血小板加速破坏。然而,抗血小板抗体检测尚未被确立为对管理决策具有临床实用性。成人ITP的自然病史和长期预后仍未完全明确。

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引用本文的文献

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Medical treatments for idiopathic thrombocytopenic purpura during pregnancy.孕期特发性血小板减少性紫癜的医学治疗。
Cochrane Database Syst Rev. 2009 Oct 7;2009(4):CD007722. doi: 10.1002/14651858.CD007722.pub2.
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Idiopathic thrombocytopenic purpura: better therapeutic responses of patients with B- or T-cell clonality than patients without clonality.特发性血小板减少性紫癜:B细胞或T细胞克隆性患者比无克隆性患者具有更好的治疗反应。
Int J Hematol. 2003 Dec;78(5):461-6. doi: 10.1007/BF02983822.
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Assessment of Equine Autoimmune Thrombocytopenia (EAT) by flow cytometry.
通过流式细胞术评估马自身免疫性血小板减少症(EAT)。
BMC Blood Disord. 2001;1(1):1. doi: 10.1186/1471-2326-1-1.