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目的探讨慢性粒单细胞白血病(chronic myelomonocytic leukemia,CMML)患者的临床特征。方法按WHO新标准回顾性分析16例CMML患者的诊断、治疗和生存情况。结果CMML的临床特点主要有:中老年男性居多;多数表现为乏力脾大;实验室检查外周血白细胞均增高,以单核细胞增高为主,乳酸脱氢酶升高;骨髓增生活跃到极度活跃,有病态造血或/和嗜酸嗜碱细胞增多;中性碱性磷酸酶积分低;免疫表型CD13+CD14+CD56+;染色体荧光原位杂交分析BCR-ABL(-)。所有患者中位生存时间为20个月余,CMML-Ⅰ平均生存时间50个月余,CMML-Ⅱ平均生存时间18个月余;同时采用MDAPS评分,结果显示低危组的平均生存时间60个月余,中危1组30个月余,中危2组17个月余,高危组1个月余,其中1例中危患者于确诊后3年余转为急性粒单细胞白血病。结论采用WHO新标准来诊断CMML更能体现患者的临床特征和提示预后,与MDAPS评分标准共同使用能更好评估疾病程度。
Objective To investigate the clinical features of patients with chronic myelomonocytic leukemia (CMML). Methods According to the new WHO criteria, the diagnosis, treatment and survival of 16 patients with CMML were retrospectively analyzed. Results The main clinical features of CMML were as follows: the majority of middle-aged men, most of which showed weak splenomegaly. The peripheral blood leucocytes in the laboratory were increased, mainly mononuclear cells increased, lactate dehydrogenase increased, myeloproliferation was extremely active , With pathological hematopoiesis or / and eosinophilic basophils; low neutral alkaline phosphatase; immunophenotype CD13 + CD14 + CD56 +; chromosomal fluorescence in situ hybridization analysis of BCR-ABL (-). The mean survival time of CMML-Ⅰ was more than 50 months, and the mean survival time of CMML-Ⅱ was 18 months. The mean survival time of the low-risk group was 60 More than a month, the intermediate risk group of more than 30 months, two groups of more than 17 months in the risk of high-risk group more than 1 month, of which 1 case of critically ill patients after the diagnosis of more than 3 years to acute myelomonocytic leukemia. Conclusion The new WHO criteria for the diagnosis of CMML can better reflect the clinical features of patients and suggest the prognosis, and MDAPS score can be used together to better assess the degree of disease.