急性早幼粒细胞白血病早期死亡危险因素剖析.docVIP

急性早幼粒细胞白血病早期死亡危险因素剖析 　　[摘要] 目的 探讨急性早幼粒细胞白血病患者早期死亡危险因素。 方法 分析我院42例APL患者发病时的白细胞计数、血小板计数、纤维蛋白原含量等特点，并比较APL患者的免疫分型、融合基因及基因突变等因素。 结果 42例APL患者中，6例出现早期死亡，早期死亡率14.3%。其中4例死于脑出血，1例死于弥漫性血管内凝血并多脏器功能衰竭，1例死于心肌梗死。死亡组患者白细胞计数显著高于完全缓解组，血小板及纤维蛋白原计数均较完全缓解组患者低，5例为CD2+，5例为CD34和（或）HLA-DR+，4例存在FT3-ITD基因突变，与完全缓解组比较差异有统计学意义（P0.05）。 结论 高白细胞血症、低血小板计数、低纤维蛋白原、CD2+、CD34和（或）HLA-DR+、FT3-ITD基因突变的急性早幼粒细胞白血病患者早期死亡率高。 　　[关键词] 急性早幼粒细胞白血病；早期死亡；免疫表型；融合基因；基因突变 　　[中图分类号] R733.71 [文献标识码] A [文章编号] 1673-9701（2015）16-0015-03 　　[Abstract] Objective To investigate risk factors for early death in acute promyelocytic leukemia patients. Methods The clinical characteristics including leucocyte counts at onset，platelet count and fibrinogen content of 42 patients with APL were evaluated. The factors of immunophenotype， genes fusion as well as gene mutations were compared. Results Among the 42 patients with APL， 6 cases were observed early death with the early mortality rate of 14.3%. To be specific， 4 deaths were attributable to cerebral hemorrhage， whereas the other two were linked with disseminated intravascular coagulation complicated with multiple organ failure and myocardial infarction respectively. The leukocyte counts of dead patients were significantly more than complete remission group. At the same time， the blood platelet counts and the fibrinogen counts were all less than those of complete remission patients. 5 patients were confirmed as CD2+， 5 cases were CD34 and/or HLA-DR+， and FLT3-ITD gene mutation was found in 4 subjects. Difference between the two groups was statistically significant（P0.05）. Conclusion Acute promyelocytic leukemia patients with leukocytosis， low platelet count， hypofibrinogenemia， CD2+， CD34 and/or HLA-DR+ as well as FLT3-ITD gene mutation are at an increased risk of early death in the risk factor analysis. 　　[Key words] Acute promyelocytic leukemia； Early death； Immunophenotype； Fusiongene； Gene mutations 　　急性早幼粒细胞白血病（acute promyelocytic leu-kemia，APL）是急性髓系白血病的特殊类型，大量的白细胞分化停滞在