Auewarakul C U, Huang S, Yimyam M, Boonmoh S
Department of Medicine, Division of Hematology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Acta Haematol. 2006;116(2):114-9. doi: 10.1159/000093641.
Little evidence exists regarding the prognostic impact of the major BCR-ABL gene variants (e13a2 and e14a2) in chronic myeloid leukemia (CML) patients diagnosed and treated in the developing Asian countries. In this study, 139 Thai CML patients were followed for a median period of 3 years (range 18-43 months). Clinical presentations of both BCR-ABL gene variant groups (73% e14a2+ and 27% e13a2+) were similar, although e14a2+ patients tended to be older (42 vs. 37 years) and had higher white blood cell counts than e13a2+ patients. The majority of patients in both groups presented with Sokal stage 2-3 (score >0.8) and were categorized as Hasford's intermediate- to high-risk groups (score >780). All patients received oral chemotherapy and 13% underwent allogeneic stem cell transplantation. None received oral tyrosine kinase inhibitors. In the conventional chemotherapy group, the overall survival (OS) rate was slightly better in e14a2+ than in e13a2+ patients (p = n.s.). The median survival in e14a2+ and e13a2+ patients who did not receive stem cell transplantation was 49 and 33 months, respectively (p = n.s.). The type of blastic crisis in e14a2+ and e13a2+ patients was similar, being predominantly myeloid. In conclusion, CML patients in Thailand, despite being much younger, had a comparable OS with those in the Western countries, with no different OS between e14a2+ and e13a2+ patients. Future studies should focus on the impact of novel oral BCR-ABL tyrosine kinase inhibitors on the outcome of Thai CML patients with different BCR-ABL gene variants.
关于主要的BCR-ABL基因变异（e13a2和e14a2）对在亚洲发展中国家诊断和治疗的慢性髓性白血病（CML）患者预后的影响，目前证据较少。在本研究中，对139例泰国CML患者进行了为期3年的中位随访（范围18 - 43个月）。两个BCR-ABL基因变异组（73% e14a2+和27% e13a2+）的临床表现相似，尽管e14a2+患者往往年龄较大（42岁对37岁），且白细胞计数高于e13a2+患者。两组中的大多数患者表现为索卡尔2 - 3期（评分>0.8），并被归类为哈斯福德中高危组（评分>780）。所有患者均接受口服化疗，13%接受了异基因干细胞移植。无人接受口服酪氨酸激酶抑制剂治疗。在传统化疗组中，e14a2+患者的总生存率（OS）略高于e13a2+患者（p =无统计学意义）。未接受干细胞移植的e14a2+和e13a2+患者的中位生存期分别为49个月和33个月（p =无统计学意义）。e14a2+和e13a2+患者的急变期类型相似，主要为髓系。总之，泰国的CML患者尽管年龄要小得多，但与西方国家的患者有相当的总生存率，e14a2+和e13a2+患者之间的总生存率没有差异。未来的研究应关注新型口服BCR-ABL酪氨酸激酶抑制剂对不同BCR-ABL基因变异的泰国CML患者结局的影响。
