骨髓增生异常综合征向急性白血病转化的研究

Study on the transformation from myelodysplastic syndromes into acute leukemias

目的 探讨骨髓增生异常综合征 (MDS)向急性白血病 (AL)转化 (转白 )的规律。方法 动态观察 15 1例MDS患者的转白情况及转白患者 (postMDS AL)的临床表现、血常规、骨髓象、染色体核型、细胞免疫表型及治疗反应、预后特点。结果 8年 7个月内 ,15 1例MDS患者转白 2 1例(13 91% ) ,中位转白时间 5 (1～ 2 3)个月。RA、RAEB、RAEB t间转白率差异无显著性 ,逐步转白和直接转白病例均可见。影响MDS转白的因素有 :年龄≤ 40岁 ;三系血细胞减少 ;骨髓原始细胞比例≥0 15 0 ;复杂染色体核型异常 ;联合化疗。postMDS AL 2 1例皆为急性髓系白血病 (AML) ,以M2 、M4 和M5为主。其中 2例 (9.5 2 % )有白血病细胞浸润表现 ;10例 (47.6 2 % )外周血白细胞计数减少 ,14例 (6 6 .6 7% )外周血中性粒细胞计数减少 ,14例 (6 6 6 7% )骨髓增生活跃或明显活跃。 8例 (47.0 6 % )转白前后异常染色体核型发生了改变。骨髓单个核细胞髓系不成熟抗原CD3 3 (49.83± 2 4.5 0 ) %、CD13 (36 38±33.84) % ,单核细胞相关抗原CD14 (38.5 0± 2 4.6 0 ) %及早期造血细胞标志抗原CD3 4(34 .6 7± 30 5 9) % ,也可共表达淋巴细胞系抗原CD5、CD7、CD9、CD19。postMDS AML的诱导化疗完全缓解率为 31 2 5 % ,中位生存时间 6 (1～

Objective To study the patterns of transformation from myelodysplastic syndromes(MDS) into acute leukemias(AL). Methods Leukemic transformation of MDS patients was dynamically followed up and the clinical manifestations, peripheral blood and bone marrow pictures, karyotypes, immunophenotypes, response to treatment and prognosis of post MDS acute leukemia(postMDS AL) were observed. Results During the past eight year and seven months, 21(13.91%) of 151 MDS patients progressed to overt leukemia with a median interval of 5(1～21)months. There were no significant differences among the rates of leukemia from RA, RAEB and RAEB t groups. The transformation was developed either gradually or rapidly. There were five parameters related to the leukemic transformation:under 40 years of age, pancytopenia, more than 0.15 blasts in bone marrow, at least two types of abnormal karyotype and combined chemotherapy. All of the 21 post MDS AL were acute myeloid leukemia (AML); and most of them were M 2, M 4 and M 5. Two(9.52%) post MDS AML developed extramedullary infiltration. Leukopenia was found in 47.62% of patients. Two third of the patients, whose bone marrows were generally hypercellular, showed neutropenias. After evolving into AML, 8(47.06%) patients developed abnormal karyotypes. High expression of immature myeloid antigens, including CD 33 (49.83±24.50)%, CD 13 (36.38±33 84)%, monocytic antigen CD 14 (38.50±24.60)%, and stem cell marker CD 34 (34.67±30.59)% were found on bone marrow mononuclear cells of post MDS AML cases. In some cases, lymphoid antigens, such as CD 5, CD 7, CD 9 and CD 19 were coexisted with myeloid antigens. A low complete remission rate(31.25%) and short survival duration with median survival of 6(1～28) months were found in patients with post MDS AML treated by induction therapy. Conclusion MDS was at high risk of evolving into AML, either gradually or rapidly. Patients with post MDS AML had specific biologic features and worse prognoses.