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首页> 外文期刊>British Journal of Haematology >Efficacy and safety of pegylated-interferon α-2a in myelofibrosis: A study by the FIM and GEM French cooperative groups
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Efficacy and safety of pegylated-interferon α-2a in myelofibrosis: A study by the FIM and GEM French cooperative groups

机译：聚乙二醇干扰素α-2a在骨髓纤维化中的功效和安全性：FIM和GEM法国合作组织的研究

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Myeloproliferative neoplasm-related myelofibrosis is associated with cytopenic or proliferative phases, splenomegaly and constitutional symptoms. Few effective treatments are available and small series suggested that interferon could be an option for myelofibrosis therapy. We performed a retrospective study of pegylated-interferon α-2a (Peg-IFNα-2a) therapy in myelofibrosis. Sixty-two patients treated with Peg-IFNα-2a at 17 French and Belgian centres were included. Responses were determined based on the criteria established by the International Working Group for Myelofibrosis Research and Treatment. Mean follow-up was 26 months. Sixteen of 25 anaemic patients (64%) (eight concomitantly receiving recombinant erythropoietin) achieved a complete response and transfusion-independence was obtained in 5/13 patients (38·5%). Constitutional symptoms resolved in 82% of patients. All five leucopenic patients normalized their leucocyte counts, whereas a normal platelet count was obtained in 5/8 thrombocytopenic patients. Splenomegaly was reduced in 46·5% of patients, and complete resolution of thrombocytosis and leucocytosis were observed in 82·8% and 68·8% of patients, respectively. Side effects (mostly haematological) were mainly of grade 1-2. The only factor independently associated with treatment failure was a spleen enlargement of more than 6 cm below the costal margin. In conclusion, Peg-IFNα-2a induced high response rates with acceptable toxicity in a large proportion of patients with primary and secondary myelofibrosis, especially in early phases.
机译：骨髓增生性肿瘤相关的骨髓纤维化与细胞减少或增生期，脾肿大和体质症状有关。几乎没有有效的治疗方法，小系列研究表明干扰素可能是骨髓纤维化治疗的一种选择。我们进行了聚乙二醇化干扰素α-2a（Peg-IFNα-2a）治疗骨髓纤维化的回顾性研究。包括在法国的17个和比利时的中心接受Peg-IFNα-2a治疗的62例患者。根据国际骨髓纤维化研究和治疗工作组确定的标准确定反应。平均随访26个月。 25例贫血患者中有16例（64％）（其中8例同时接受重组促红细胞生成素）获得了完全缓解，5/13例患者中获得了输血独立性（38·5％）。体质症状在82％的患者中得到解决。所有五名白细胞减少症患者的白细胞计数均正常化，而5/8血小板减少症患者的血小板计数正常。脾肿大减少了46·5％的患者，并且分别在82·8％和68·8％的患者中观察到血小板增多和白细胞增多的完全消退。副作用（主要是血液病）主要为1-2级。与治疗失败独立相关的唯一因素是脾脏增大到肋缘以下6 cm以上。总之，Peg-IFNα-2a在大部分原发性和继发性骨髓纤维化患者中，特别是在早期阶段，可诱导高反应率和可接受的毒性。

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