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再生障碍性贫血骨髓移植后发生的致命性自身免疫性全血细胞减少症。

Fatal autoimmune pancytopenia following bone marrow transplantation for aplastic anaemia.

作者信息

De Lord C, Marsh J C, Smith J G, Singer C R, Gordon-Smith E C

机构信息

Department of Haematology, St George's Hospital, London, UK.

出版信息

Bone Marrow Transplant. 1996 Jul;18(1):237-9.

PMID:8832026
Abstract

We report a case of autoimmune pancytopenia 10 months after allogeneic bone marrow transplantation (BMT) for severe aplastic anaemia (SAA). The autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenic purpura (ITP) were refractory to conventional immunosuppressive therapy which included steroids, azathioprine, vincristine and intravenous immunoglobulin. Splenectomy led to a recovery of the thrombocytopenia but the haemolysis continued despite further immunosuppressive therapy. Four months after the onset of haemolysis granulocyte-specific antibodies were detected. The patient subsequently received total lymph node irradiation (TLI) with a peripheral blood stem cell transplant (PBSCT) from his original donor, but died 9 days later from cerebral aspergillosis. The severe nature of autoimmune cytopenias and their lack of response to conventional treatment following allogeneic BMT is discussed further.

摘要

我们报告了1例严重再生障碍性贫血(SAA)患者在接受异基因骨髓移植(BMT)10个月后发生自身免疫性全血细胞减少症的病例。该患者的自身免疫性溶血性贫血(AIHA)和免疫性血小板减少性紫癜(ITP)对包括类固醇、硫唑嘌呤、长春新碱和静脉注射免疫球蛋白在内的传统免疫抑制治疗无效。脾切除术后血小板减少症有所恢复,但尽管进一步进行了免疫抑制治疗,溶血仍持续存在。溶血发作4个月后检测到粒细胞特异性抗体。该患者随后接受了来自其原供体的外周血干细胞移植(PBSCT)及全淋巴结照射(TLI),但9天后死于脑曲霉病。本文进一步讨论了异基因BMT后自身免疫性血细胞减少症的严重性及其对传统治疗的无反应性。

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