Ikeda Y, Yoshinaga K, Iki S, Ohbayashi Y, Urabe A
Division of Hematology, Kanto Teishin Hospital.
Rinsho Ketsueki. 1993 Sep;34(9):1022-6.
A 32-year-old man visited Kanto Teishin Hospital complaining of general fatigue in May, 1992. He had been diagnosed as having paroxysmal nocturnal hemoglobinuria since 1980, because of brownish urine in the morning. He received blood transfusion in 1980. In 1983, he was treated with medication. There was no remarkable improvement, however, and he stopped coming to the hospital. When he was admitted to our hospital, hemolytic anemia and hemosiderinuria were noticed. Sucrose hemolysis test and acidified-serum lysis test (Ham test) were both positive. Positive rates of decay accelerating factor and CD59 were 38.8% (control 100%) and 45.4% (control 100%), respectively. His diagnosis was thus confirmed. Bone marrow was slightly hypocellular, and erythroid cells were relatively hyperplastic (M/E ratio 0.68). The oral administration of iron and oxymetholone was not effective for anemia. He was treated with daily subcutaneous administration of recombinant human erythropoietin (EPO, 3,000U/body/day). His hemoglobin level increased from 7.5g/dl to 12.0g/dl in 4 weeks, and general fatigue disappeared. Since he had concurrent chronic hepatitis C, alpha-interferon was also administered and his hemoglobin level is now controlled between 10 and 11g/dl. This case suggests that EPO can be useful for treating hemolytic anemia, even though erythroid cells in the bone marrow are hyperplastic.
一名32岁男性于1992年5月因全身乏力就诊于关东帝新医院。自1980年起，因其晨起尿液呈褐色，他被诊断为阵发性夜间血红蛋白尿。1980年他接受了输血治疗。1983年，他接受了药物治疗。然而，病情并无明显改善，他也不再来医院就诊。当他入住我院时，发现有溶血性贫血和含铁血黄素尿。蔗糖溶血试验和酸化血清溶血试验（汉姆试验）均呈阳性。衰变加速因子和CD59的阳性率分别为38.8%（对照组为100%）和45.4%（对照组为100%）。由此确诊。骨髓细胞轻度减少，红系细胞相对增生（M/E比值为0.68）。口服铁剂和羟甲烯龙对贫血无效。他接受了每日皮下注射重组人促红细胞生成素（EPO，3000U/人/天）的治疗。4周内他的血红蛋白水平从7.5g/dl升至12.0g/dl，全身乏力症状消失。由于他同时患有慢性丙型肝炎，还给予了α干扰素治疗，目前他的血红蛋白水平控制在10至11g/dl之间。该病例表明，即使骨髓中的红系细胞增生，EPO对治疗溶血性贫血也可能有效。
