BCR-ABL阳性急性髓系白血病的临床回顾性分析

Retrospective Study of Ph^+/BCR-ABL^+ Acute Myelogenous Leukemia

目的:探讨Ph^+/BCR-ABL^+急性髓系白血病患者的临床表现、实验室检查特征及预后。方法:收集本院自2007年7月至2015年12月共5例急性髓系白血病伴Ph^+/BCR-ABL^+病例,分析其临床、实验室特征及长期生存情况。结果:5例原发Ph^+急性髓系白血病患者,中位年龄39岁。2例脾肿大。5例均可检测到BCR-ABL^+,其中2例伴有其他分子生物学异常。4例Ph^+中1例为复杂核型,另有1例未检测到Ph染色体。3例行传统化疗联合TKI与异基因造血干细胞移植巩固治疗,目前均存活;1例患者行单纯化疗,6个月后死亡;1例患者行伊马替尼联合化疗存活至今。结论:Ph^+/BCR-ABL^+急性髓系白血病行酪氨酸激酶抑制剂(Tyrosine Kinase Inhibitor,TKI)联合化疗,序贯异基因造血干细胞移植治疗可以延长患者的生存期。

Objective： To investigate the clinical manifestation,features of laboratorial examination results and prognosis of patients with Ph＋/BCR-ABL＋ acute myelogenous leukemia（ AML）. Methods： The clinical data of 5 AML patients with Ph＋/BCR-ABL＋ admitted in Department of Hematology of Chinese PLA general hospital from July 2007 to May 2015 were collected and their clinical characteristics,laboatorial examination results and long-term survival were analyzed.Results： The median age of 5 cases was 39 years old,and 2 cases with splenomegaly. All the cases were assayed for BCR-ABL fusion gene,and 2 of them were accompanied with other molecular abnormalities. In 4 cases,Ph chromosome was not found in one case,and one was with complex karyotype. 3 cases still are live till now and are treated by traditional chemotherapy combined with TKI,and consolidated by allo-HSCT. One case treated by traditional chemotherapy survived for 6 months. And one case treated by traditional chemotherapy combined with TKI survives till to now. Conclusion： The survival time of Ph＋/BCR-ABL＋ acute myelogenous leukemia is improved by the traditional chemotherapy combined with TKI and the consolidation with allo-HSCT.