Miura Y, Ueda M, Kondou Y, Yamasaki H, Takami A, Sugimori N, Saito M, Nakao S, Shiohara S, Saito K, Matsuda T
Third Department of Medicine, Kanazawa University School of Medicine.
Rinsho Ketsueki. 1997 Jun;38(6):526-31.
A 28-year-old male was diagnosed as aplastic anemia in 1983. He maintained on corticosterone with a large transfusion requirement for being resistant to other therapies, and combined with hemochromatosis at 20-year-old. In February 1994, he was admitted to the hospital for consideration of BMT. Echocardiogram was normal on admission. He was transplanted with bone marrow from his HLA-matched MLC negative sister following contained of TLI (7.5 Gy) and CY 50 mg/kg for four days on March 10 1994. Disturbance of consciousness appeared, an echocardiogram showed severe pericardial effusion on day 1 after BMT. He was diagnosed cardiac tamponade, pericardiocentesis was done immediately and 100 ml pericardial effusion was removed. Transiently he became alert, however, irreversible cardiac arrest occurred on day 2. Postmortem examination revealed thickened left ventricles with intramyocardial hemorrhage. It seems necessary to reduce CY, or substitute it with anti-thymocyte globulin (ATG) or TBI etc. for BMT in aplastic anemia accompanied by hemochromatosis.
一名28岁男性于1983年被诊断为再生障碍性贫血。由于对其他治疗有抵抗性,他一直依靠皮质类固醇维持治疗,输血需求量大,并且在20岁时并发血色素沉着症。1994年2月,他因考虑进行骨髓移植入院。入院时超声心动图正常。1994年3月10日,在接受7.5 Gy全身照射(TLI)和连续4天给予50 mg/kg环磷酰胺(CY)预处理后,他接受了来自其HLA匹配且混合淋巴细胞培养阴性的姐姐的骨髓移植。骨髓移植后第1天出现意识障碍,超声心动图显示严重心包积液。他被诊断为心脏压塞,立即进行了心包穿刺,抽出100 ml心包积液。他一度恢复清醒,但在第2天发生了不可逆的心脏骤停。尸检显示左心室增厚伴心肌内出血。对于伴有血色素沉着症的再生障碍性贫血患者进行骨髓移植时,似乎有必要减少环磷酰胺的用量,或者用抗胸腺细胞球蛋白(ATG)或全身照射(TBI)等替代环磷酰胺。