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[溶血性贫血合并白塞病和骨髓增生异常综合征]

[Hemolytic anemia complicated with Behçet's disease and myelodysplastic syndrome].

作者信息

Chyuma Y, Utsunomiya A, Saito T, Hanada S, Nishimata H, Arima T

机构信息

Second Department of Internal Medicine, Faculty of Medicine, Kagoshima University.

出版信息

Rinsho Ketsueki. 1992 Mar;33(3):333-7.

PMID:1578637
Abstract

A rare case of hemolytic anemia complicated with Behçet's disease and myelodysplastic syndrome (MDS) is described. A 41-year old woman suffering from hemolytic anemia was admitted in July of 1988 with right lower abdominal pain and a high fever. Her anemia was first pointed out in 1962 (at age 15), and diagnosed as hemolytic anemia in 1977 by a full hematological examination showing erythro-hyperplasia in bone marrow, Coomb's test was negative and corticosteroid therapy failed to improve her anemia. She had also been suffering from recurrent stomatitis and genital ulcer since the delivery of her first baby in July, 1972. Barium enema was performed and revealed a simple deep ulcer at the terminal ileum. Bone marrow examination showed morphological abnormalities of granulocytic and erythrocytic series. We thereby diagnosed her illness as incomplete Behçet's disease and MDS associated with hemolytic anemia. She was treated by ubenimex, blood transfusion and intravenous alimentation with discontinuing oral intake, and there was a satisfactory improvement in pancytopenia and ulcer.

摘要

本文描述了一例罕见的溶血性贫血合并白塞病和骨髓增生异常综合征(MDS)的病例。一名41岁患有溶血性贫血的女性于1988年7月因右下腹痛和高热入院。她的贫血于1962年(15岁时)首次被发现,1977年经全面血液学检查显示骨髓红系增生,抗人球蛋白试验阴性且皮质类固醇治疗未能改善其贫血,从而被诊断为溶血性贫血。自1972年7月她首次分娩后,还一直患有复发性口腔炎和生殖器溃疡。进行了钡剂灌肠检查,发现回肠末端有一个单纯的深部溃疡。骨髓检查显示粒细胞系和红细胞系存在形态学异常。因此,我们将她的疾病诊断为不完全性白塞病和与溶血性贫血相关的MDS。她接受了乌苯美司治疗、输血和静脉营养支持并停止口服摄入,全血细胞减少和溃疡情况有了令人满意的改善。

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