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骨髓移植后溶血性尿毒症综合征

Hemolytic uremic syndrome following bone marrow transplantation.

作者信息

Juckett M, Perry E H, Daniels B S, Weisdorf D J

机构信息

Bone Marrow Transplantation Program, University of Minnesota, Minneapolis.

出版信息

Bone Marrow Transplant. 1991 May;7(5):405-9.

PMID:2070153
Abstract

Bone marrow transplant (BMT) is increasingly being offered as therapy for certain hematologic and other advanced malignancies. We present one representative patient in detail and summarize data from a series of 10 patients who received a BMT and, as a late complication, developed a hemolytic uremic syndrome (HUS). All patients presented with the triad of microangiopathic hemolytic anemia, renal insufficiency and thrombocytopenia from 30 to 875 days after BMT, and despite aggressive supportive management, plasma exchange, IgG administration and/or ex vivo staphylococcal protein A column plasma treatment, eight of 10 patients died from complications related to HUS between 11 to 139 days after diagnosis. In contrast to other reports of HUS after BMT, the present series of patients is heterogeneous with respect to underlying diagnosis, type of BMT (allogeneic or autologous), pretransplant conditioning regimen, presence of graft-versus-host disease, and use of cyclosporin. Additionally, nine of 10 patients were in clinical remission at the time of diagnosis of HUS, and six of 10 patients had achieved a complete recovery following BMT. The cause of HUS in this series of patients is probably multifactorial and related to the intensive pretransplant conditioning regimen.

摘要

骨髓移植(BMT)越来越多地被用作某些血液系统及其他晚期恶性肿瘤的治疗方法。我们详细介绍了一名代表性患者,并总结了一系列10例接受BMT治疗的患者的数据,这些患者在晚期出现了溶血性尿毒症综合征(HUS)。所有患者在BMT后30至875天出现微血管病性溶血性贫血、肾功能不全和血小板减少三联征,尽管进行了积极的支持治疗、血浆置换、静脉注射免疫球蛋白和/或体外葡萄球菌蛋白A柱血浆治疗,但10例患者中有8例在诊断后11至139天死于与HUS相关的并发症。与其他关于BMT后HUS的报道不同,本系列患者在基础诊断、BMT类型(同种异体或自体)、移植前预处理方案、移植物抗宿主病的存在以及环孢素的使用方面存在异质性。此外,10例患者中有9例在诊断HUS时处于临床缓解期,10例患者中有6例在BMT后已完全康复。本系列患者发生HUS的原因可能是多因素的,与强化的移植前预处理方案有关。

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