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干燥综合征/红斑狼疮重叠综合征患者的纯红细胞再生障碍性贫血

Pure red cell aplasia in a Sjögren's syndrome/lupus erythematosus overlap patient.

作者信息

Mavragani Clio P, Vlachaki Eythymia, Voulgarelis Michalis

机构信息

Department of Pathophysiology, School of Medicine, National University of Athens, Greece.

出版信息

Am J Hematol. 2003 Apr;72(4):259-62. doi: 10.1002/ajh.10302.

DOI:10.1002/ajh.10302
PMID:12666137
Abstract

Hematological complications of systemic lupus erythematosus usually include anemia of chronic disease and peripheral destruction of blood cells. We describe the case of a young woman with Sjögren's syndrome'lupus erythematosus overlap, mother of infant with congenital heart block, complicated by pure red cell aplasia. The patient was asymptomatic until the onset of severe anemia. A serum inhibitor of erythropoiesis was detected before the onset of immunosuppression. Bone marrow examination showed a low CD4:CD8 ratio, an immune defect possibly linked with the unrestrained production of antibodies against erythroid progenitor cells.

摘要

系统性红斑狼疮的血液学并发症通常包括慢性病贫血和血细胞的外周破坏。我们描述了一名患有干燥综合征-红斑狼疮重叠综合征的年轻女性病例,她是一名患有先天性心脏传导阻滞婴儿的母亲,并发了纯红细胞再生障碍。该患者在严重贫血发作前无症状。在免疫抑制开始前检测到一种红细胞生成血清抑制剂。骨髓检查显示CD4:CD8比值低,这是一种可能与针对红系祖细胞的抗体不受控制的产生相关的免疫缺陷。

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Pure red cell aplasia in a Sjögren's syndrome/lupus erythematosus overlap patient.干燥综合征/红斑狼疮重叠综合征患者的纯红细胞再生障碍性贫血
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引用本文的文献

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Anaemia in systemic lupus erythematosus: from pathophysiology to clinical assessment.系统性红斑狼疮中的贫血:从病理生理学到临床评估
Ann Rheum Dis. 2006 Feb;65(2):144-8. doi: 10.1136/ard.2005.041673. Epub 2005 Aug 3.