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获得性无巨核细胞性血小板减少症迅速进展为再生障碍性贫血。

Rapid progression of acquired amegakaryocytic thrombocytopenia to aplastic anemia.

作者信息

King J A, Elkhalifa M Y, Latour L F

机构信息

Department of Pathology, University of South Alabama, Mobile 36617-2293, USA.

出版信息

South Med J. 1997 Jan;90(1):91-4. doi: 10.1097/00007611-199701000-00024.

DOI:10.1097/00007611-199701000-00024
PMID:9003837
Abstract

Acquired amegakaryocytic thrombocytopenia is a rare disorder characterized by severe thrombocytopenia and selective, marked decrease or absence of megakaryocytes. Although immunosuppressive therapy (prednisone and/or antithymocyte globulin) has been shown to induce remissions in a subset of patients, most patients do not respond, and progression to aplastic anemia occurs in some cases. We report a case of acquired amegakaryocytic thrombocytopenia which, despite aggressive immunosuppressive treatment, rapidly progressed to aplastic anemia. Clinical, laboratory, and immunologic features of our patient's case are described and compared to those of the previously reported six cases that progressed from amegakaryocytic thrombocytopenia to aplastic anemia.

摘要

获得性无巨核细胞性血小板减少症是一种罕见的疾病,其特征为严重血小板减少以及巨核细胞选择性显著减少或缺乏。尽管免疫抑制疗法(泼尼松和/或抗胸腺细胞球蛋白)已被证明可使部分患者缓解,但大多数患者并无反应,且在某些情况下会进展为再生障碍性贫血。我们报告一例获得性无巨核细胞性血小板减少症病例,该病例尽管接受了积极的免疫抑制治疗,仍迅速进展为再生障碍性贫血。描述了我们患者病例的临床、实验室和免疫学特征,并与先前报道的6例从无巨核细胞性血小板减少症进展为再生障碍性贫血的病例进行了比较。

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