Heis-Vahidi-Fard N, Forberg E, Eichinger S, Chott A, Lechner K, Gisslinger H
Department of Internal Medicine I, Division of Haematology and Blood Coagulation, Währinger Gürtel 18-20, 1090 Vienna, Austria.
Ann Hematol. 2001 Feb;80(2):79-82. doi: 10.1007/s002770000231.
It has been proposed that interferon-gamma (IFN) inhibits collagen synthesis in myeloproliferative disorders through an inhibitory effect on PDGF and TGF-beta. We therefore evaluated the role of IFN-gamma on bone marrow fibrosis in idiopathic myelofibrosis (IMF). After a 3-month observation period, nine patients (five female, four male), median age 64 years (range 43-72 years), received 3 x 3 mU IFN-gamma/week over 6 months and were monitored after withdrawal of IFN-gamma for further 3 months. Three out of nine patients have completed the study according to the protocol. Six patients had to be withdrawn from IFN-gamma due to the following reasons: bacterial infection (three patients), splenic infarction or deterioration of splenomegaly (one patient, each) and refusal to continue IFN-gamma (one patient). Results from seven patients treated for at least 8 weeks were considered measurable. Leukopenia, initially present in one of the evaluated patients, deteriorated during IFN-gamma treatment. This patient died during the observation period shortly after withdrawal of the therapy as a result of septicemia. Transfusion-dependent anemia, initially observed in two of the evaluated patients, deteriorated during the IFN-gamma treatment. Bone marrow fibrosis increased in three patients, whereas it remained unchanged in another and improved in a further patient. Splenomegaly improved in two patients but deteriorated markedly in one. Taking these observations together, four patients had disease progression during IFN-gamma treatment, two had stable disease and one could be qualified as a partial responder. According to these data IFN-gamma cannot be considered as a treatment option for patients with IMF.
有人提出，干扰素-γ（IFN）通过对血小板衍生生长因子（PDGF）和转化生长因子-β（TGF-β）的抑制作用，抑制骨髓增殖性疾病中的胶原合成。因此，我们评估了IFN-γ在原发性骨髓纤维化（IMF）中的骨髓纤维化作用。经过3个月的观察期后，9例患者（5例女性，4例男性），中位年龄64岁（范围43 - 72岁），在6个月内每周接受3×3 mU IFN-γ治疗，并在停用IFN-γ后再监测3个月。9例患者中有3例已按方案完成研究。6例患者因以下原因不得不停用IFN-γ：细菌感染（3例）、脾梗死或脾肿大恶化（各1例）以及拒绝继续使用IFN-γ（1例）。对至少治疗8周的7例患者的结果进行了评估。白细胞减少最初出现在1例评估患者中，在IFN-γ治疗期间恶化。该患者在观察期内停用治疗后不久因败血症死亡。输血依赖型贫血最初在2例评估患者中观察到，在IFN-γ治疗期间恶化。3例患者的骨髓纤维化加重，而另1例保持不变，还有1例有所改善。脾肿大在2例患者中有所改善，但在1例患者中明显恶化。综合这些观察结果，4例患者在IFN-γ治疗期间病情进展，2例病情稳定，1例可被视为部分缓解者。根据这些数据，IFN-γ不能被视为IMF患者的一种治疗选择。
