Lévy V, Bourgarit A, Delmer A, Legrand O, Baudard M, Rio B, Zittoun R
Service d'Hématologie, Hôtel-Dieu, Paris, France.
Am J Hematol. 1996 Dec;53(4):239-41. doi: 10.1002/(SICI)1096-8652(199612)53:4<239::AID-AJH5>3.0.CO;2-Z.
Peripheral cytopenias are common in patients with agnogenic myeloid metaplasia (AMM). They are an important cause of morbidity and mortality, and their treatment is difficult. We report on 4 patients with AMM and severe cytopenia treated with danazol (400-600 mg/ day). Three of them became independent of red blood cell (RBC) transfusion, while the other had a slight reduction in RBC requirement. In addition, correction of thrombocytopenia and disappearance of splenomegaly were observed in 1 and 2 patients, respectively. No side effects were observed. In our experience, danazol appears effective and safe in the subset of patients with AMM whose disease is mainly characterized by bone-marrow failure. These data warrant further studies to evaluate this treatment and explore its mechanism of action.
外周血细胞减少在原发性骨髓化生(AMM)患者中很常见。它们是发病和死亡的重要原因,并且其治疗困难。我们报告了4例接受达那唑(400 - 600毫克/天)治疗的AMM和严重血细胞减少患者。其中3例不再需要红细胞(RBC)输血,而另1例对RBC的需求略有减少。此外,分别在1例和2例患者中观察到血小板减少的纠正和脾肿大的消失。未观察到副作用。根据我们的经验,达那唑在以骨髓衰竭为主要特征的AMM患者亚组中似乎有效且安全。这些数据值得进一步研究以评估这种治疗方法并探索其作用机制。