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骨髓增生异常综合征(MDS),是造血干细胞异常的克隆性疾病。儿童发病率为3.4/10万,目前尚无特效疗法。我科对18例MDS患儿,根据临床不同分型采取不同方案治疗,现报道如下。 资料与结果 一、临床资料 MDS患儿18例,女2例,男16例;年龄8月～16岁,平均6.3岁。病人均系原发型初治MDS患儿,经临床、血象、骨髓涂片、骨髓染色体检查及祖细胞培养确诊。FAB分型如下,难治型贫血(RA)12例,难治型贫血伴原始细胞增多(RAEB)2例,转化中的难治型贫血伴原始细胞增多(RAEB-T)2例,慢性粒单核细胞白血病(CMML)2例。
Myelodysplastic syndrome (MDS), is a clonal disease of hematopoietic stem cell abnormalities. The incidence of children is 3.4 / 100,000, there is no effective therapy. Our department of 18 cases of MDS children, according to different clinical options to take different programs, are reported below. Materials and Results First, clinical data 18 cases of children with MDS, 2 females, 16 males; aged August to 16 years, mean 6.3 years. The patients were all primary MDS children with primary diagnosis by clinical, blood, bone marrow smears, bone marrow chromosome examination and progenitor cell culture. The FAB classification was as follows: 12 cases of refractory anemia (RA), 2 cases of refractory anemia with blasts (RAEB), 2 cases of transformed refractory anemia with blasts (RAEB-T) 2 cases of monocytic leukemia (CMML).