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作者用大剂量强的松龙治疗阵发性夜间血红蛋白尿(paroxysmal nocturnal hemoglobinuria,PNH),评价其疗效。方法患者19例,男13、女6例,年龄13～56(中数26)岁。均有溶血史和血红蛋白尿。诊断根据 Ham试验阳性和出现血红蛋白尿。给予强的松龙60mg,隔日1次,贫血加重时输红细胞。对治疗3个月以上的患者进行疗效评价。疗效判断标准为:①显效:血红蛋白增加,不需要输血;②进步:血红蛋白增加但需输血;③无效:血红蛋白不增加。显效的患者逐渐减少强的松龙用量
The authors used high-dose prednisolone to treat paroxysmal nocturnal hemoglobinuria (PNH) to evaluate its efficacy. Methods 19 patients, 13 males and 6 females, aged 13 to 56 (median 26) years old. Have a history of hemolysis and hemoglobinuria. The diagnosis is based on Ham test positive and hemoglobinuria. Give prednisolone 60mg, every other day 1, red blood cells lose when the anemia increases. The treatment of patients over 3 months efficacy evaluation. Efficacy criteria: ① markedly: hemoglobin increased, do not need blood transfusion; ② progress: hemoglobin but need to be transfused; ③ invalid: hemoglobin does not increase. Effective patients gradually reduce the dosage of prednisolone