Papageorgiou S G, Castleton A, Bloor A, Kottaridis P D
Department of Haematology, Royal Free and University College London Medical School, London, UK.
Bone Marrow Transplant. 2006 Dec;38(11):721-7. doi: 10.1038/sj.bmt.1705516. Epub 2006 Oct 2.
Idiopathic myelofibrosis (IMF) is a clonal disorder resulting from the proliferation of aberrant hematopoietic stem cells. Conventional treatment is unsatisfactory, and with the exception of supportive blood transfusions, none of the standard therapies have been shown to confer a survival advantage. Allogeneic stem cell transplantation represents the only treatment modality with proven curative potential. Myeloablative conditioning regimens are associated with high transplant-related mortality, particularly in the elderly, making most patients with IMF ineligible for this treatment. Strategies using reduced intensity conditioning regimes have allowed application of allogeneic transplantation to a broader range of patients and a number of recent reports have demonstrated potential efficacy.
原发性骨髓纤维化(IMF)是一种由异常造血干细胞增殖引起的克隆性疾病。传统治疗效果不理想,除了支持性输血外,尚无标准疗法显示能带来生存优势。异基因干细胞移植是唯一被证实具有治愈潜力的治疗方式。清髓性预处理方案与高移植相关死亡率相关,尤其是在老年患者中,这使得大多数IMF患者不适合这种治疗。采用减低强度预处理方案的策略已使异基因移植能够应用于更广泛的患者群体,最近的一些报告已证明其潜在疗效。