Myelodysplastic syndromes

被引：374

作者：

Ades, Lionel ^{[1
,2
]}

Itzykson, Raphael ^{[1
,2
]}

Fenaux, Pierre ^{[1
,2
]}

机构：

[1] Hop St Louis, AP HP, Hematol Serv, F-75475 Paris, France

[2] Univ Paris 07, F-75475 Paris, France

来源：

LANCET | 2014年 / 383卷 / 9936期

关键词：

ACUTE MYELOID-LEUKEMIA; PROGNOSTIC SCORING SYSTEM; STEM-CELL TRANSPLANTATION; BONE-MARROW-TRANSPLANTATION; COLONY-STIMULATING FACTOR; TRANSFUSION-DEPENDENT PATIENTS; CONVENTIONAL CARE REGIMENS; IRON CHELATION-THERAPY; RANDOMIZED PHASE-II; REGULATORY T-CELLS;

D O I：

10.1016/S0140-6736(13)61901-7

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Myelodysplastic syndromes are clonal marrow stem-cell disorders, characterised by ineffective haemopoiesis leading to blood cytopenias, and by progression to acute myeloid leukaemia in a third of patients. 15% of cases occur after chemotherapy or radiotherapy for a previous cancer; the syndromes are most common in elderly people. The pathophysiology involves cytogenetic changes with or without gene mutations and widespread gene hypermethylation at advanced stages. Clinical manifestations result from cytopenias (anaemia, infection, and bleeding). Diagnosis is based on examination of blood and bone marrow showing blood cytopenias and hypercellular marrow with dysplasia, with or without excess of blasts. Prognosis depends largely on the marrow blast percentage, number and extent of cytopenias, and cytogenetic abnormalities. Treatment of patients with lower-risk myelodysplastic syndromes, especially for anaemia, includes growth factors, lenalidomide, and transfusions. Treatment of higher-risk patients is with hypomethylating agents and, whenever possible, allogeneic stem-cell transplantation.

引用

页码：2239 / 2252

页数：14

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