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与胸腺瘤相关的纯红细胞再生障碍:胸腺切除术后贫血完全缓解

Pure Red Cell Aplasia Associated with Thymolipoma: Complete Anaemia Resolution following Thymectomy.

作者信息

Ferreira David, Ponraj Royston, Yeung Adrian, de Malmanche Jillian

机构信息

Conjoint Associate Lecturer, University of New South Wales, Sydney, Australia.

Medical Department, Medical Registrar, Liverpool Hospital, Elizabeth St., Liverpool, NSW 2170, Australia.

出版信息

Case Rep Hematol. 2018 Oct 9;2018:8627145. doi: 10.1155/2018/8627145. eCollection 2018.

Abstract

Pure red cell aplasia is an uncommon cause of anaemia rarely associated with thymoma. A combination of immunosuppressive therapy and thymectomy offers a potential cure. Thymectomy alone rarely results in anaemia resolution. A seventy-three-year-old male with Klinefelter syndrome presented with progressively increasing shortness of breath and anaemia. Serological testing supported primary bone marrow pathology, and a bone marrow biopsy was performed. A pure red cell aplasia was seen on bone marrow examination, and computed tomography of the chest demonstrated a thymoma. Thymectomy was performed, and histology revealed a thymolipoma. Complete anaemia resolution was achieved following thymectomy alone. This suggests that thymomas may directly mediate immune dysregulation resulting in erythroid precursor destruction.

摘要

纯红细胞再生障碍性贫血是一种罕见的贫血病因,很少与胸腺瘤相关。免疫抑制治疗和胸腺切除术联合应用有可能治愈该病。单独进行胸腺切除术很少能使贫血得到缓解。一名患有克兰费尔特综合征的73岁男性,出现进行性加重的气短和贫血症状。血清学检测支持原发性骨髓病变,遂进行了骨髓活检。骨髓检查发现纯红细胞再生障碍性贫血,胸部计算机断层扫描显示有胸腺瘤。实施了胸腺切除术,组织学检查显示为胸腺脂肪瘤。仅通过胸腺切除术后贫血就完全缓解。这表明胸腺瘤可能直接介导免疫失调,导致红系前体细胞破坏。

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