List A F, Kummet T D, Kerr D M
Department of Internal Medicine, Veterans Administration Medical Center, Phoenix, Arizona 85723.
Leuk Res. 1990;14(4):321-6. doi: 10.1016/0145-2126(90)90158-6.
Acute myelofibrosis is a rare myeloproliferative syndrome characterized by bone marrow proliferation of atypical megakaryocytes, poor response to conventional leukemic therapy, and a fulminant clinical course. Because alpha interferon exhibits potent antiproliferative effects against megakaryocyte progenitors and human fibroblast cell lines, we treated two patients with acute myelofibrosis or the related syndrome of acute myelodysplasia with myelofibrosis with recombinant human interferon alpha-2a. Patient 1 received a 12-week course of interferon alpha (1-6 x 10(6) IU/d) after failure of two cytarabine-based chemotherapy regimens. Interferon administration resulted in prompt improvement in symptoms, stabilization of leukocyte count, and a reduction in circulating blast forms. Primary treatment with interferon (1-3 x 10(6) IU/d x 4 weeks) in patient 2 produced complete hematologic recovery with restoration of marrow cellularity and reduced marrow fibrosis. Our findings suggest that interferon alpha may have significant activity in the treatment of patients with acute myelofibrosis.
急性骨髓纤维化是一种罕见的骨髓增殖综合征，其特征为非典型巨核细胞在骨髓中增殖、对传统白血病治疗反应不佳以及临床病程凶险。由于α干扰素对巨核细胞祖细胞和人成纤维细胞系具有强大的抗增殖作用，我们用重组人干扰素α-2a治疗了两名急性骨髓纤维化患者或伴有骨髓纤维化的急性骨髓发育异常相关综合征患者。患者1在两种基于阿糖胞苷的化疗方案失败后，接受了为期12周的干扰素α疗程（1 - 6×10⁶国际单位/天）。给予干扰素后症状迅速改善、白细胞计数稳定，循环原始细胞形态减少。患者2采用干扰素（1 - 3×10⁶国际单位/天×4周）进行初始治疗后实现了完全血液学缓解，骨髓细胞增多，骨髓纤维化减轻。我们的研究结果表明，α干扰素在治疗急性骨髓纤维化患者方面可能具有显著活性。
