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血栓性血小板减少性紫癜对血浆输注和血浆置换无反应,但对脾切除术有反应。

Thrombotic thrombocytopenic purpura unresponsive to plasma infusion and plasma exchange, but responsive to splenectomy.

作者信息

Sturgess A D, Chong B H

出版信息

Scand J Haematol. 1986 Oct;37(4):319-22. doi: 10.1111/j.1600-0609.1986.tb02320.x.

DOI:10.1111/j.1600-0609.1986.tb02320.x
PMID:3787182
Abstract

A 60-yr-old female presented with typical thrombotic thrombocytopenic purpura (TTP). She remained in coma with frequent seizures for 1 wk, with persisting severe thrombocytopenia and microangiopathic haemolytic anaemia, despite treatment with prednisolone, plasma exchange, fresh frozen plasma, sulphinpyrazone and dipyridamole. Splenectomy induced haematological improvement within 1 d, there was cessation of fitting after 2 d, and full neurological recovery ensued over 3 wk. Laboratory studies did not reveal the presence of a platelet-aggregating factor (PAF), stated to be present in some two-thirds of cases. While plasma exchange and plasma infusion are beneficial in many cases, splenectomy appears still to be of value in unresponsive disease.

摘要

一名60岁女性表现出典型的血栓性血小板减少性紫癜(TTP)。尽管接受了泼尼松龙、血浆置换、新鲜冷冻血浆、磺吡酮和双嘧达莫治疗,但她仍昏迷1周,频繁癫痫发作,持续存在严重血小板减少和微血管病性溶血性贫血。脾切除术后1天内血液学状况得到改善,2天后癫痫发作停止,3周内神经功能完全恢复。实验室检查未发现血小板聚集因子(PAF)的存在,据说约三分之二的病例中存在该因子。虽然血浆置换和血浆输注在许多病例中有益,但脾切除术对于难治性疾病似乎仍有价值。

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