醋酸甲羟孕酮与纯合子镰状细胞病

Medroxyprogesterone acetate and homozygous sickle-cell disease.

作者信息

De Ceulaer K, Gruber C, Hayes R, Serjeant G R

出版信息

Lancet. 1982 Jul 31;2(8292):229-31. doi: 10.1016/s0140-6736(82)90320-8.

Abstract

The haematological and clinical effects of medroxyprogesterone acetate in homozygous sickle-cell (SS) disease were assessed in a 2-year controlled crossover trial completed by 23 patients. Haematological indices remained steady during the placebo phase, but during the medroxyprogesterone-acetate phase fetal haemoglobin, total haemoglobin, red-cell mass, and red-cell survival rose significantly, and reticulocytes, irreversibly-sickled-cell counts, and total bilirubin fell significantly. Painful crises were significantly less frequent during the medroxyprogesterone-acetate than the placebo phase. These results are compatible with an inhibition of in-vivo sickling in patients with SS disease during medroxyprogesterone-acetate treatment. The mechanisms of such an effect require further study.

摘要

在一项由23名患者完成的为期2年的对照交叉试验中，评估了醋酸甲羟孕酮对纯合子镰状细胞（SS）病的血液学和临床影响。在安慰剂阶段，血液学指标保持稳定，但在醋酸甲羟孕酮阶段，胎儿血红蛋白、总血红蛋白、红细胞量和红细胞存活率显著上升，而网织红细胞、不可逆镰状细胞计数和总胆红素显著下降。与安慰剂阶段相比，醋酸甲羟孕酮阶段的疼痛性危象明显减少。这些结果与醋酸甲羟孕酮治疗期间SS病患者体内镰状化受到抑制相一致。这种作用的机制需要进一步研究。