以难治性单系血细胞减少为早期表现的骨髓增生异常综合征——附13例报道

Myelodysplastic Syndrome with Early Presentation of Refractory Monolineage Cytopenia----with Report of 13 Cases

为揭示难治性单系血细胞减少与骨髓增生异常综合征(MDS)之间关系,对13例以难治性单系血细胞减少为早期表现的MDS进行了回顾性分析。结果如下:(1)10年中以顽固性单系血细胞减少为早期表现的MDS患者共13例,占我院确诊219例MDS患者的5.9％,其中粒细胞减少者4例,红细胞减少者4例,血小板减少者5例。(2)患者从出现单系血细胞减少至确诊为MDS的平均时间为(48.5±55.3)月,其中粒细胞减少、红细胞减少和血小板减少的平均时间分别为(12.5±9.5)月,(53.8±54.6)月和(59.2±65.5)月。(3)13例患者有以下特点:①大红细胞增多,骨髓中红系中、晚幼红细胞比例升高;②偶有一过性个别细胞病态造血表现;③红细胞减少和血小板减少者均转为RA和RAS亚型,而粒细胞减少者大多转为RAEB;④部分患者有自身抗体。结论:部分难治性单系血细胞减少实质上为一种早期MDS的表现。

To find the relationship between myelodysplastic syndrome ( MDS) and refractory monolineage cytopenia,thirteen cases of MDS with early presentation of monolineage refractory cytopenia were analyzed retrospectively.The results were as follows: (l)The percentage of 13 cases with refractory monolineage cytopenia were 5.9% of the total 219 MDS patients in the past 10 years.(2)The median time of patients with monlineage cytopenia to MDS diagnosed was (48.5 + 55.3) months.The median times from monolineage cytopenia to MDS diagnosed for patients with neutropenia,erythrocytopenia and thrombocytopenia were (12.5+9.5) months,(53.8+54.6) months and (59.2 + 65.5 ) months,respectively.(3)The common characteristics of 13 cases were as follows: (a) the macrocytic erythrocytes in peripheral blood and the percentage of intermediate and late erythroblast in bone marrow were increased; (b) occasionally few cells with dysplasia could be found; (c) all patients with erythrocytopenia and thrombocytopenia transformed to RA and RAS while the most of patients with neutropenia transformed to RAEB subtype; (d) autoantibody could be found in part of the patients.It is concluded that some of refractory monolineage cytopenias in essence are the early states of MDS.