摘要
目的：探讨MLL-AF10阳性急性白血病患者的临床特征及预后。方法回顾性分析6例MLL-AF10阳性急性白血病患者的临床资料，结合文献明确MLL-AF10阳性急性白血病患者的临床特征及预后。结果6例患者中男4例，女2例，中位年龄为19.5岁，其中5例患者以发热起病，4例患者起病时WBC＜10×10^9/L，FAB分型M55例，M41例，初诊时MLL-AF10融合基因水平（实时定量PCR法）为0.23%～22.60%，4例患者均同时合并WT1基因突变，流式细胞术显示为髓系表达。5例可评估患者经常规化疗1个疗程均获得完全缓解，后均行异基因造血干细胞移植（allo-HSCT）。6例患者中2例复杂核型者死亡（1例死于诱导治疗期败血症，1例移植后未植入死亡），5例移植患者中4例获得长期生存。结论 MLL-AF10阳性急性白血病患者多为年轻男性，FAB分型以M5、M4居多，常以发热起病，初诊时白细胞计数低，起病时MLL-AF10融合基因水平低，常合并WT1基因突变，常规化疗具有较高的缓解率，但易复发，复杂核型者预后差，allo-HSCT有可能改善其预后。
Objective To analyze the clinical features and prognosis of acute leukemia patients with the mixed lineage leukemia （MLL） gene rearrangements AF10 positive. Methods 6 cases with MLL-AF10 positive were analyzed retrospectively, related literatures were reviewed to clarify MLL-AF10 patients’clinical features and prognosis. Results The median age of 6 cases was 19.5 years old, 5 patients with fever onset, the onset white blood cells of 4 patients were less than 10×10^9/L. 5 cases were as M5 and 1 case M4 according to FAB classification, the level of fusion gene（RQ-PCR）was 0.23%-22.60%when diagnosed, 4 cases had concomitant WT1 gene mutation, flow cytometry disclosed myeloid phenotype. Of 5 evaluated patients achieved the first complete remission after conventional chemotherapy, 2 cases of complex karyotype died, one case died of sepsis in induction, another died from failing of transplantation. 4 out of 5 transplant recipients gained long term survival. Conclusions The MLL-AF10 positive patients were mostly young men, the majority FAB classification was M5 or M4, often onset with fever, low white blood cells and low level of fusion gene, usually associated with WT1 mutation. Conventional chemotherapy produced a high response rate, but easy to relapse, while the complex karyotype had a poor prognosis, allo-HSCT may have the potential to improve the prognosis of MLL-AF10 positive patients.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2015年第10期840-843,共4页
Chinese Journal of Hematology
