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克罗恩病-加拿大综合征患者的再生障碍性贫血。

Aplastic Anemia in a Patient with Cronkhite-Canada Syndrome.

机构信息

Division of Hematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, Japan.

Department of Transfusion Medicine, Saga University Hospital, Japan.

出版信息

Intern Med. 2021;60(10):1601-1605. doi: 10.2169/internalmedicine.6468-20. Epub 2021 May 15.

Abstract

Cronkhite-Canada syndrome (CCS) is a rare polyposis disorder accompanied by alopecia and onychodystrophy. A 63-year-old man with a history of CCS and repeated embolism developed progressive thrombocytopenia and mild anemia. Laboratory testing, a bone marrow examination, and magnetic resonance imaging of the spine resulted in a diagnosis of concurrent aplastic anemia (AA). Paroxysmal nocturnal hemoglobinuria (PNH)-type cells were detected in a peripheral blood specimen. In addition, human leukocyte antigen (HLA) included DRB115:01 and DRB115:02. Mesalazine was discontinued in consideration of possible drug-induced pancytopenia. Immunosuppressive therapy ameliorated both the gastrointestinal symptoms of CCS and pancytopenia. A common autoimmune abnormality might underlie both CCS and AA.

摘要

克罗恩-加拿大综合征(CCS)是一种罕见的伴有脱发和甲营养不良的息肉病。一位 63 岁男性患有 CCS 病史和反复栓塞,出现进行性血小板减少和轻度贫血。实验室检查、骨髓检查和脊柱磁共振成像诊断为再生障碍性贫血(AA)。在外周血标本中检测到阵发性睡眠性血红蛋白尿症(PNH)样细胞。此外,人类白细胞抗原(HLA)包括 DRB115:01 和 DRB115:02。考虑到可能的药物诱导全血细胞减少,停止使用美沙拉嗪。免疫抑制疗法改善了 CCS 的胃肠道症状和全血细胞减少症。共同的自身免疫异常可能是 CCS 和 AA 的基础。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3eb7/8188031/985af5e89e7a/1349-7235-60-1601-g001.jpg

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