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一位异基因骨髓移植受者溶血性尿毒症综合征的肝脏表现。

Hepatic manifestation of hemolytic uremic syndrome in an allogeneic bone marrow transplant recipient.

作者信息

Böcher W O, Schirmacher P, Löhr H F, Kolbe K, Wanitschke R, Meyer zum Büschenfelde K H

机构信息

Department of Internal Medicine, Hospital of the Johannes Gutenberg University Mainz, Germany.

出版信息

Z Gastroenterol. 1995 Sep;33(9):543-5.

PMID:8525659
Abstract

A 33-year-old woman presented 42 days after allogeneic bone marrow transplantation for acute monocytic leukemia (AML, FAB M5) with persistent thrombocytopenia, acute renal failure and Coombs negative hemolytic anemia. In the absence of the disseminated intravascular coagulation the diagnosis of hemolytic uremic syndrome due to immunosuppression with cyclosporin A was supposed. Because cessation of cyclosporin A and therapeutic infusions of fresh frozen plasma had failed, plasmaseparation therapy was started on day 79 after bone marrow transplantation. While hemolytic anemia improved during ongoing plasmaseparations the patient developed cholestatic liver failure due to hepatic manifestation of HUS. The histological lesions of liver involvement in thrombotic microangiopathies are discussed and a review of the literature is presented.

摘要

一名33岁女性在接受异基因骨髓移植治疗急性单核细胞白血病(AML,FAB M5)42天后出现持续性血小板减少、急性肾衰竭和抗人球蛋白试验阴性的溶血性贫血。在无弥散性血管内凝血的情况下,推测为环孢素A免疫抑制所致的溶血性尿毒症综合征。由于停用环孢素A和输注新鲜冷冻血浆治疗均无效,在骨髓移植后第79天开始进行血浆置换治疗。在持续血浆置换过程中,溶血性贫血有所改善,但患者因溶血性尿毒症综合征的肝脏表现而发展为胆汁淤积性肝衰竭。本文讨论了血栓性微血管病累及肝脏的组织学病变,并对相关文献进行了综述。

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Hepatic manifestation of hemolytic uremic syndrome in an allogeneic bone marrow transplant recipient.一位异基因骨髓移植受者溶血性尿毒症综合征的肝脏表现。
Z Gastroenterol. 1995 Sep;33(9):543-5.
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