DA或HA方案治疗难治、复发性阵发性睡眠性血红蛋白尿症的初步观察

目的　观察DA[柔红霉素 (DNR) 4 0mg第 1,2天 ,2 0mg第 3天 ;阿糖胞苷 (Ara C) 10 0mg d ,共 5d]或HA[高三尖杉酯碱 (HHT) 2～ 3mg d ,共 5d ;Ara C 10 0mg d ,共 5d]联合化疗方案治疗难治、复发性阵发性睡眠性血红蛋白尿症 (PNH)患者的疗效及不良反应。方法　 3例使用DA方案 ,5例应用HA方案治疗难治、复发性PNH患者 ,分析疗效及不良反应。结果 8例患者化疗均有效 ,所有患者肾上腺糖皮质激素的用量较化疗前减少 6 6 %～ 10 0 % (中位数 75 % )。 6例检查PNH克隆患者治疗后 5例PNH细胞比例明显减少 ,1例无变化。 6例溶血指标明显好转。 8例患者经治疗后 2例进步 ,6例明显进步。 7例依赖输血者中 6例脱离输血。未发现严重的不良反应。结论DA或HA方案是治疗难治、复发性PNH较为安全、有效的方法。

Preliminary study of DA or HA regimen chemotherapy for the treatment of refractory and relapsed paroxysmal nocturnal hemoglobinuria

OBJECTIVE: To observe the efficacy and side effect of DA/HA regimen chemotherapy for the treatment of refractory and relapsed paroxysmal nocturnal hemoglobinuria (PNH). METHODS: Eight patients with refractory and relapsed PNH were treated with DA/HA regimen chemotherapy. Three patients were treated with DA (DNR 40 mg/d, i.v.drip, the first and the second day; 20 mg/d, i.v.drip, the third day; Ara-C 100 mg/d, i.v.drip, for 5 days) and 5 patients with HA (HHT 2 - 3 mg/d, i.v.drip, for 5 days; Ara-C 100 mg/d, i.v.drip, for 5 days). RESULTS: All the 8 patients responded well: the PNH clone was diminished in five patients. Hemolysis was remitted in 6 cases. Five patients showed improvement in hematological parameters. The dosage of corticosteroid was decreased in all of them. No serious side effect was revealed. CONCLUSION: DA/HA regimen chemotherapy was safe and effective for refractory and relapsed PNH patients.