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影响血栓性血小板减少性紫癜患者血浆治疗反应的因素。

Factors affecting the response to plasma therapy in thrombotic thrombocytopenic purpura.

作者信息

Koyama T, Kakishita E, Nagai K

机构信息

Second Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Japan.

出版信息

Jpn J Med. 1989 Jan-Feb;28(1):16-21. doi: 10.2169/internalmedicine1962.28.16.

Abstract

Factors affecting the response to plasma therapy (plasma exchange and plasma infusion) were studied in four cases of thrombotic thrombocytopenic purpura (TTP) treated in our hospital. Plasma therapy led to recovery from advanced TTP, but three of the patients exhibited chronic relapse. The longer the period from onset, the more marked was the progression of TTP, from the first stage (thrombocytopenia alone) to the second stage in which thrombocytopenia was accompanied by microangiopathic hemolytic anemia (MHA), and the third stage in which neurological abnormalities appeared. Platelet thrombi, which are thought to be the primary pathogenetic feature, probably enlarge leading to stenosis of the vessels during the progression of TTP. Although the severity of TTP in the early stages could be judged from two markers, LDH and the platelet count, it was impossible to determine the severity of advanced TTP by these two markers. However, the severity of advanced TTP could be judged by including the grade and period of neurological abnormalities. The dose of plasma needed to induce recovery was small in the early stage, but as the period from onset lengthened, the dose had to be increased. Though the early platelet thrombi might be easily eliminated by plasma infusion, larger and well-established platelet thrombi might not be lysed even by massive doses of plasma.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

在我院接受治疗的4例血栓性血小板减少性紫癜(TTP)患者中,研究了影响血浆治疗(血浆置换和血浆输注)反应的因素。血浆治疗使晚期TTP患者康复,但其中3例患者出现慢性复发。从发病开始的时间越长,TTP的进展越明显,从第一阶段(仅血小板减少)发展到第二阶段(血小板减少伴有微血管病性溶血性贫血,MHA),再到第三阶段(出现神经异常)。血小板血栓被认为是主要的致病特征,在TTP进展过程中可能会增大,导致血管狭窄。虽然早期TTP的严重程度可以通过乳酸脱氢酶(LDH)和血小板计数这两个指标来判断,但无法通过这两个指标确定晚期TTP的严重程度。然而,晚期TTP的严重程度可以通过纳入神经异常的分级和时间来判断。诱导康复所需的血浆剂量在早期较小,但随着发病时间延长,剂量必须增加。虽然早期的血小板血栓可能很容易通过血浆输注消除,但即使使用大剂量血浆,较大且形成良好的血小板血栓也可能无法溶解。(摘要截断于250字)

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