Human platelet antigens and primary immune thrombocytopenia.
Human platelet antigens and primary immune thrombocytopenia.
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DOI：
10.1016/j.bjhh.2017.02.008
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发表时间：
2017-04
影响因子：
--
通讯作者：
Castro V
中科院分区：
文献类型：
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作者：
Castro V
原发性免疫性血小板减少性紫癜（ITP）是一种获得性免疫介导的疾病，其特征是短暂或持续的血小板计数降低（< 100× 109/L），在没有其他潜在原因的情况下影响儿童和成人。1，2血小板计数低是由于抗血小板自身抗体破坏血小板所致，与血小板生成不足（也与这些自身抗体有关）和T细胞免疫失调有关（图1）。2约70%的ITP患者血清中存在自身抗体，主要针对血小板表面糖蛋白（GP），如GP Ⅱ b-Ⅲ a、GP Ⅰ b-IX和GP Ⅰ a-Ⅱ a。2，3 ITP的估计发病率为5.8例/100，000名年轻人，性别分布相似，1.6例/100，000名中年人，女性发病率较高（1.9名女性：1名男性）。这种情况可以根据持续时间分为新诊断（少于3个月），持续（3-12个月）和慢性（超过12个月）。1、2儿童和成人的临床特征通常不同。在儿童时期，ITP通常突然发作，通常在病毒感染后1-2周或接种疫苗（通常为麻疹、腮腺炎和风疹-MMR疫苗接种）后长达6周，无论治疗如何，约70-80%的病例在几周内自发恢复。在成年人中，这种疾病有一个阴险的发病，没有
Primary immune thrombocytopenic purpura (ITP) is an acquired immune-mediated disorder characterized by transient or persistent decreased platelet count (< 100× 109/L) that affects children and adults in the absence of other underlying causes. 1, 2 The low platelet count results from platelet destruction by antiplatelet autoantibodies associated to causes such as insufficient platelet production, which is also related to these autoantibodies, and T cell immune dysregulation (Figure 1). 2The presence of the autoantibodies may be demonstrated in the serum of approximately 70% of ITP patients, usually directed against platelet surface glycoproteins (Gp), such as Gp IIb-IIIa, Gp Ib-IX and Gp Ia-IIa. 2, 3 ITP has an estimated incidence of 5.8 cases in 100,000 young individuals with similar distribution between genders, and 1.6 cases in 100,000 middle aged individuals with higher rates among women (1.9 females: 1 male). This condition may be classified according to duration as newly diagnosed (less than 3 months), persistent (3–12 months) and chronic (more than 12 months). 1, 2 T he clinical features are usually different in children and adults. In childhood, ITP usually has an abrupt onset, often starting 1–2 weeks after a viral infection or up to six weeks after vaccinations (generally the measles, mumps and rubella–MMR vaccination) with recovery being spontaneous in around 70–80% of the cases within a few weeks regardless of therapy. In adults, the disease has an insidious onset, with no