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[自身免疫性溶血性贫血激素治疗期间发生重型再生障碍性贫血]

[Development of severe aplastic anemia during steroid therapy for autoimmune hemolytic anemia].

作者信息

Yoshida Kozue, Murai Kazunori, Hamada Hiroyuki, Sato Akiyoshi, Miyairi Yasurou, Wano Yuji

机构信息

Department of Hematology, Iwate Prefectural Isawa Hospital.

Department of Hematology, Iwate Prefectural Central Hospital.

出版信息

Rinsho Ketsueki. 2022;63(3):171-176. doi: 10.11406/rinketsu.63.171.

Abstract

A 50-year-old male patient was admitted for close monitoring of anemia (hemoglobin level, 5.0 g/dl). Autoimmune hemolytic anemia (AIHA) of warm type was diagnosed based on the elevated reticulocyte and bone marrow erythroblast counts, elevated indirect bilirubin level, serum haptoglobin level below the detection limit, and positive direct Coombs test result. The patient responded to prednisolone 60 mg/day (1.0 mg/kg); however, pancytopenia was observed during gradual dose tapering and maintenance therapy. The bone marrow showed remarkable hypoplastic findings, and magnetic resonance imaging scans of the thoracolumbar spinal cord showed an overgrowth of the adipose tissue. Thus, the patient was diagnosed with aplastic anemia (AA) stage 4. He was successfully treated with a combination of immunosuppressive therapy (anti-thymocyte globulin +cyclosporine), which allowed him to reduce his dependence on transfusions. However, the direct Coombs test result remained positive even after hematopoietic recovery. Aplastic anemia following AIHA treatment is extremely rare and has not been reported previously.

摘要

一名50岁男性患者因贫血(血红蛋白水平为5.0 g/dl)入院接受密切监测。根据网织红细胞和骨髓成红细胞计数升高、间接胆红素水平升高、血清触珠蛋白水平低于检测限以及直接抗人球蛋白试验结果呈阳性,诊断为温抗体型自身免疫性溶血性贫血(AIHA)。患者对泼尼松龙60 mg/天(1.0 mg/kg)有反应;然而,在逐渐减量和维持治疗期间观察到全血细胞减少。骨髓显示出明显的增生低下表现,胸腰椎脊髓的磁共振成像扫描显示脂肪组织过度生长。因此,该患者被诊断为4期再生障碍性贫血(AA)。他接受免疫抑制治疗(抗胸腺细胞球蛋白+环孢素)联合治疗后获得成功,这使他减少了对输血的依赖。然而,即使造血恢复后,直接抗人球蛋白试验结果仍为阳性。AIHA治疗后发生再生障碍性贫血极为罕见,此前未见报道。

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