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