Fei Xin-Hong, Wu Shu-Lan, Sun Rui-Juan, Zhou Jia-Rui, Wang Jing-Bo, Wang Tong, Liu Hong-Xing, Wang Hui, Tong Chun-Rong, Wu Tong, Lu Dao-Pei
Beijing Dao-Pei Hospital, Beijing, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2012 Jun;20(3):545-8.
This study was purposed to analyze the characteristics of morphology, immunology, cytogenetic and molecular biology of leukemia cells in 12 AML patients with Ph(+) and their correlation with survival of patients. 12 patients with Ph(+) AML were diagnosed according to diagnostic criteria of WHO and existence of t(9;22) (q34;q11) or t(9;22) abnormality, meanwhile no evidence of CML chronic phase was observed. The results showed that 8 out of 12 cases were confirmedly diagnosed to be AML by morphologic and immunophenotypic examination, 4 cases were diagnosed as myeloid and B lymphocytic mixed acute leukemia. The Ph chromosome was detected in 10 cases by chromosome analysis at the first time of diagnosis, and some of the cases had coexistence of complex chromosome and/or normal karyotype. BCR-ABL transcript was detected in all 12 cases, including 7 cases with b3a2, 1 case with b2a2, 1 case with b2a2 variants, 2 cases with e1a2 and 1 case with e18a2. The 12 cases all got complete remission after chemotherapy and/or gleevec treatment, out of them 3 cases received chemotherapy and gleevec treatment, but 2 cases died; 9 cases received allogeneic hematopoietic stem-cell transplantation (allo-HSCT), 1 case died from relapse, among them 1 case died from transplant complications. The median survival was 24 (8 - 80) months, the overall survival of 3 years was (51.4 ± 17.7)%. It is concluded that the Ph(+) AML is a acute myelogenous leukemia with poor prognosis, but long-term survival may be achieved with HSCT as quick as after complete remission from gleevec and chemotherapy treatment. Meanwhile, the detection of BCR-ABL gene and it variants may be give more opportunity for diagnose and treatment, which can be used as routine screening for newly diagnosed leukemia.
本研究旨在分析12例Ph(+)急性髓系白血病（AML）患者白血病细胞的形态学、免疫学、细胞遗传学及分子生物学特征及其与患者生存的相关性。12例Ph(+) AML患者根据世界卫生组织（WHO）诊断标准及t(9;22)(q34;q11)或t(9;22)异常确诊，同时未观察到慢性粒细胞白血病（CML）慢性期证据。结果显示，12例中8例经形态学和免疫表型检查确诊为AML，4例诊断为髓系和B淋巴细胞混合急性白血病。初诊时染色体分析10例检测到Ph染色体，部分病例存在复杂染色体并存和/或正常核型。12例均检测到BCR-ABL转录本，其中b3a2型7例，b2a2型1例，b2a2变异型1例，e1a2型2例，e18a2型1例。12例经化疗和/或格列卫治疗后均获完全缓解，其中接受化疗和格列卫治疗3例，2例死亡；接受异基因造血干细胞移植（allo-HSCT）9例，1例死于复发，其中1例死于移植并发症。中位生存期为24（8 - 80）个月，3年总生存率为（51.4 ± 17.7）%。结论：Ph(+) AML是一种预后不良的急性髓系白血病，但格列卫和化疗治疗完全缓解后尽早行HSCT可能实现长期生存。同时，检测BCR-ABL基因及其变异可为诊断和治疗提供更多机会，可作为新诊断白血病的常规筛查手段。
