不典型慢性髓系白血病九例临床分析

Clinical Analysis of Atypical Chronic Myeloid Leukemia:Report of 9 Cases

目的探讨不典型慢性髓系白血病(aCML)的发生、发展及转归。方法对我院9例明确诊断为aC-ML病人的临床资料进行回顾性分析。结果9例病人的中位年龄为66岁;均有白细胞升高,中度贫血6例,红系增生不良7例,明显的粒系病态造血5例,巨核细胞增生不良7例,骨髓纤维化2例;脾大6例,肝大1例,感染3例,出血6例,均需要输血;主要症状为贫血;化疗效果较差;其中3例患者在确诊为aCML前有骨髓增生异常综合征(MDS)病史多年,最长达16年,最短为2年;发现罕见染色体异常,并呈多样性。结论少数MDS最终的转化趋势为aCML。

Objective To study the ocurrance, development and turnover of atypical chronic myeloid leukemia （aCML）. Methods The clinical data of 9 patients finally diagnosed as having aCML were analyzed retrospectively. Results The mean age of 9 patients was 66 years old ; all had increased white blood cells （WBC） ; moderate anemia was presented in 6 cases （66. 7% ）, erythriod dysplasia in 7 cases （77.8%）, obvious granulocytic dyshaematopoiesis in 5 （55.6%）, megakaryocytic dysplasia in 7 （77.8%）, myelofibrosis in 2 （22. 2% ）, splenomegaly in 6 （66.7%）, hepatomegaly in 1 （ 11.1% ）, infection in 3 （33.3%）, hemorrhage in 6 （66. 7% ）, all required blood transfusion. The cardinal symptom was anemia; chemotherapy was less effective; three of them had a long history of MDS （the longest up to 16 years, the shortest up to 2 years） before finally diagnosed as having aCML; rare chromosomal abnormality was noted, presenting a diversity. Conclusion The ultimate trend of a small number of myelodysplastic syndromes （MDS） is for aCML.