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A new name in thrombosis, ADAMTS13

机译:血栓形成的新名称ADAMTS13

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摘要

Thrombotic thrombocytopenic purpura (TTP) is a rapidly fatal illness, first described by Moschowitz in 1924, that is characterized by anemia, a low platelet count (thrombocytopenia), and microvas-cular thrombosis. The anemia is caused by hemolysis and is associated with sharply pointed red cell fragments, termed schis-tocytes, that look as though they might have been sliced with a knife. The thrombocytopenia is caused by platelet deposition in thrombi, which leads to tissue damage that can produce renal failure, stroke, myocardial infarction, and acute respiratory distress. TTP often attacks otherwise healthy adults, with a preference for females of childbearing age. Without treatment the mortality rate exceeds 90%, but plasma exchange therapy has reduced the death rate to approximately 25% .
机译:血栓性血小板减少性紫癜(TTP)是一种快速致命的疾病,由Moschowitz于1924年首次描述,其特征在于贫血,血小板计数低(血小板减少)和微血管血栓形成。贫血是由溶血引起的,并伴有尖锐的红细胞碎片,称为裂殖细胞,看起来好像是用刀将其切开了一样。血小板减少症是由血小板在血栓中沉积引起的,导致组织损伤,可导致肾功能衰竭,中风,心肌梗塞和急性呼吸窘迫。 TTP通常会攻击健康的成年人,偏爱育龄女性。未经治疗,死亡率超过90%,但是血浆置换疗法已将死亡率降低至约25%。

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