DEK-CAN融合基因阳性急性髓系白血病患者临床特征及预后分析

Clinical features and prognosis analysis of acute myeloid leukemia patients with DEK-CAN-positive

目的探讨DEK-CAN融合基因阳性急性髓系白血病(AML)患者的临床特征及预后。方法回顾性分析2014年8月至2018年1月郑州大学第一附属医院收治的6例DEK-CAN融合基因阳性AML患者的临床资料,总结其临床特征、治疗及转归。结果6例患者均为女性,中位年龄29岁(4~64岁)。6例患者中,原始粒细胞白血病部分分化型(M_(2))5例,急性单核细胞白血病(M5)1例。外周血白细胞数升高5例,骨髓病态造血1例,嗜碱性粒细胞增多2例。6例患者免疫表型均CD34、CD13、CD38、CD33阳性。融合基因检测示患者DEK-CAN均为阳性,NPM1突变均为阴性,3例合并FLT3-ITD突变,2例合并WT1突变。染色体核型分析示2例未见分裂象,其余均为t(6;9)。6例患者中仅1例儿童患者第1个疗程诱导化疗取得完全缓解,5例成年患者第1个疗程化疗均未达到完全缓解,且在短期内死于并发症。结论DEK-CAN融合基因阳性AML患者预后极差,初次诱导缓解率低,死亡率高。

Objective To investigate the clinical features and prognosis of patients with acute myeloid leukemia(AML)with DEK-CAN-positive.Methods The clinical data of 6 AML patients with DEK-CAN-positive admitted to the First Affiliated Hospital of Zhengzhou University from August 2014 to January 2018 were retrospectively analyzed.Their clinical features,treatment and outcomes were summarized.Results All 6 patients were female with a median age of 29 years(4-64 years).Among 6 cases,5 cases were primary granulocytic leukemia partially differentiated(M_(2))and 1 case was acute monocyte leukemia(M5).There were 5 patients with elevated count of white blood cells in peripheral blood,and 1 patient had bone marrow dysplasia,and 2 patients had basophilia.Immunophenotypic analysis showed that all 6 patients were positive for CD34,CD13,CD38 and CD33.Fusion gene detection showed that DEK-CAN was positive and NPM1 mutations were negative in 6 patients;FLT3-ITD mutation was detected in 3 patients and WT1 mutation was detected in 2 patients.Chromosomal karyotyping showed that 2 cases had no split phase and 4 cases carried t(6;9).Follow-up details were available for 6 patients,only 1 patient achieved complete remission after the first course of induction chemotherapy,and the remaining 5 patients had no complete remission after the first course of chemotherapy,and died of complications in a short time.Conclusion AML patients with DEK-CAN-positive have a very poor prognosis,low primary induced remission rate and high mortality.