Bennett John M, Kouides Peter A, Forman Stephen J
James P. Wilmot Cancer Center, Rochester, NY, USA.
Int J Hematol. 2002 Aug;76 Suppl 2:228-38. doi: 10.1007/BF03165122.
The Myelodysplastic Syndromes (MDS) represent a group of potentially acute myeloid leukemic disorders. There exists a delicate balance between increased apoptosis and proliferation of the leukemic hematopoietic stem cell that permits many patients to survive for years. When the balance shifts towards proliferation AML develops with a poor outcome for most but not all patients. I will review the latest proposals from the W.H.O. in classification, including pediatric MDS, prognostic factors and response criteria. Then I will present a strategy for the management of low risk patients with supportive care or low intensity treatment (cytokines, Immune modulation, anti-VEGF agents) and finally chemotherapy and intensive therapy with auto and allo BMT.
骨髓增生异常综合征(MDS)是一组潜在的急性髓系白血病性疾病。白血病造血干细胞的凋亡增加与增殖之间存在微妙的平衡,这使得许多患者能够存活数年。当平衡向增殖方向转变时,大多数(但并非所有)患者会发展为急性髓系白血病(AML),预后较差。我将回顾世界卫生组织(W.H.O.)在分类方面的最新提议,包括儿童MDS、预后因素和反应标准。然后我将提出一种针对低风险患者的管理策略,包括支持性护理或低强度治疗(细胞因子、免疫调节、抗血管内皮生长因子药物),最后是化疗以及自体和异基因骨髓移植的强化治疗。