伴CBFβ-MYH11阳性的急性粒-单核细胞白血病临床分析

Clinical analysis of aute myelomonocytic leukemia with CBFβ-MYH11-positive

目的探讨伴CBFβ-MYH11阳性的急性粒-单核细胞白血病(AMMo L)患者临床特点。方法回顾性分析81例初诊AMMo L中20例伴CBFβ-MYH11阳性患者的临床资料,包括发病年龄,性别,临床特征,治疗方案,疗效和预后。结果 81例AMMo L患者中伴CBFβ-MYH11阳性者占24.69%(20/81),该组男女比例为3∶1,发病中位年龄(8~70)38.5岁。初诊时中位白细胞数41.5×(1.05~138.8)109/L,大于100×109/L者占20%(4/20)。经1个疗程达完全缓解(CR)者16例(80%),2个疗程达CR者3例(15%),总CR率95%。5年RFS率及OS率分别为51.8%和51.3%。结论伴CBFβ-MYH11阳性的AMMo L患者诱导治疗CR率高,有较长生存期和无复发生存期。早期中断治疗和基因定量监测持续不转阴为疾病复发的主要原因。治疗过程中定量监测CBFβ-MYH11表达及其变化趋势对早期预测疾病发展及预后有重要意义。

Objective The study was amid to investigate the clinical characteristics of aute myelomonocytic leukemia. Methods The clinical date of 20 cases with CBFβ- MYH11 which were detected in 81 first visited AMMoL patients were analyzed retrospectively, including age, sex, clianieal characteristics, therapeutic regimen, curative effect as well as prognosis. Results The 20 cases account for 24. 69% （20/81 ） in the 81 first visited AMMoL patients, and the proportion of man and women was 3：1. The median age of the 20 patients was 38. 5（8 - 70）years old. At first visited, the median WBC count was 41.5× （1.05 - 138.80） 109/L, and WBC count 〉 100 × 109/L was found in 20% patients （4/20）. The complete remission（ CR）rate after 1 and 2 cycles of induction chemotherapy were：80% and 15% respec- tively, then the total CR was 95%. Estimated 5 - year relapse - free survival （RFS） and overall survial （OS） were 51.8% and 51.3% respectively. Conclusion Patiens with CBFβ- MYH11 have a tendency of higher CR rate,longer RFS and OS. Interruption of early treatment and gene quantitative monitoring duration dose not turn to negative are the main cause of disease recurrence. Quantitative monitoring of CBFβ-MYH11 expression in the process of treatment and its change trend have important significance for early disease development and prognosis.