Functional Unit of Haematology, AOU Careggi, University of Florence, Firenze, Italy.
Leuk Res. 2010 Dec;34(12):1576-88. doi: 10.1016/j.leukres.2010.01.018. Epub 2010 Feb 11.
Since 2002, date of publication of the previous Italian Society of Haematology (SIE) practice guidelines for management of myelodysplastic syndromes (MDS), novel disease-modifying treatments have been introduced and the SIE commissioned an update. After a comprehensive review of the medical literature published since January 2001, the Expert Panel formulated recommendations for the management of adult and paediatric MDS, graded according to the available evidence. The major updates are: first-line hypomethylating agents in patients with INT2-high-risk disease; controlled use of first-line lenalidomide in low-INT1 risk transfusion-dependent patients with 5q deletion; deferasirox in low-INT1 patients with a relevant transfusional load; first-line high-dose ESA in low-INT1 patients with Hb <10 g/dl and endogenous EPO <500 U/l; allogeneic HSCT first-line therapy for INT2- and high-risk patients <65 years without severe co morbidities.
自 2002 年发布前一版意大利血液学会 (SIE) 骨髓增生异常综合征 (MDS) 管理实践指南以来，已经引入了新的疾病修正治疗方法，因此 SIE 委托进行了更新。在全面回顾了自 2001 年 1 月以来发表的医学文献后，专家小组根据现有证据为成人和儿科 MDS 的管理制定了建议。主要更新内容包括：高危 INT2 疾病患者的一线低甲基化药物；在低 INT1 风险、依赖输血且伴有 5q 缺失的患者中，有条件地使用一线来那度胺；在低 INT1 且具有相关输血负担的患者中使用地拉罗司；低 INT1、Hb<10g/dl 且内源性 EPO<500U/l 的患者中使用一线高剂量 ESA；无严重合并症、年龄<65 岁的 INT2 和高危患者的首选异基因 HSCT 治疗方法。
