Hertenstein B, Kurrle E, Frickhofen N, Heil G, Heimpel H
Abteilung Innere Medizin III, Medizinische Universitätsklinik Ulm, Fed. Rep. Germany.
Eur J Haematol. 1990 Apr;44(4):213-9. doi: 10.1111/j.1600-0609.1990.tb00382.x.
Acute myelofibrosis is a rare and still ill-defined disease. Based on morphological observation, immunophenotyping and ultrastructural analysis, we support the assumption that acute myelofibrosis is a malignant disorder mainly of the megakaryocytic lineage and is closely related to acute megakaryocytic/blastic leukaemia. Consequently, the 11 patients reported here were treated with aggressive polychemotherapy with combinations including daunorubicin and cytosine arabinoside and 6-thioguanin or VP16-213. 4 complete remissions, 2 partial remissions and 1 minor response were observed. Duration of aplasia was not significantly prolonged. These findings indicate that the use of aggressive polychemotherapy is feasible in acute myelofibrosis and results in a significant number of remissions.
急性骨髓纤维化是一种罕见且仍未明确界定的疾病。基于形态学观察、免疫表型分析和超微结构分析,我们支持以下假设:急性骨髓纤维化是一种主要起源于巨核细胞系的恶性疾病,与急性巨核细胞/原始细胞白血病密切相关。因此,本文报道的11例患者接受了包括柔红霉素、阿糖胞苷和6-硫鸟嘌呤或VP16-213的联合强化多药化疗。观察到4例完全缓解、2例部分缓解和1例轻度反应。再生障碍期未显著延长。这些发现表明,强化多药化疗在急性骨髓纤维化中是可行的,并能带来大量缓解。