急性早幼粒细胞白血病患者早期死亡的临床分析

目的:探讨急性早幼粒细胞白血病(APL)患者早期死亡的临床特点。方法:分析早期死亡APL患者形态学、免疫分型、染色体核型和PML/RARα融合基因特点,并详细记录年龄、性别、起病时白细胞(WBC)计数、血小板(BPC)计数、血红蛋白(Hb)含量、骨髓中早幼粒白血病细胞占单个核细胞百分比(BMLP%)、纤维蛋白原浓度(Fib)等。结果:63例APL患者中13例早期死亡,早期死亡率20.63%。13例早期死亡APL中,7例起病时外周血白细胞大于10×109L-1,9例形态学上表现为M3v,8例为CD34+,8例为CD2+,9例PML/RARα融合基因BCR3亚型,与非早期死亡组相比形态学、免疫分型、染色体核型和PML/RARα融合基因特点等存在统计学差异。除1例死于维甲酸综合征外,其余12例均死于脑出血。结论:外周血高白细胞,形态学表现为M3v、CD34+、CD2+和PML/RARα融合基因BCR3亚型APL早期死亡率极高,临床预后差。

Clinical features of acute promyelocytic leukemias developing early death

Objective:To investigate the clinical features of acute promyelocytic leukemias developing early death.Methods:13 de novo acute promyelocytic leukemias developing early death hospitalized from May 2001 to Aug 2010 were enrolled in this study.All cases had outcomes available for complete blood counts,peripheral and bone marrow blast count,immunological markers,PML/RARA fusion gene type,coagulation laboratory parameters.Results:Among the 63 patients,13 cases developed early death with a early death rate of 20.63%.Out of the 13 early death patients,7 were leukocytosis with peripheral white blood cell counts of more than 10×109 L-1 at initial diagnosis,9 were morphologically diagnosed as M3v,8 were CD34+,8 were CD2+and 9 were BCR3 type of PML/RARA fusion.Except 1 case died of severe retinoic acid syndrome,the rest 12 patients were all died of intracerebral bleeding.Conclusions:CD34 expression in APL is an unique clinical feature associated with leukocytosis,atypical morphology of M3v,high expression of CD34 and CD2,and the S-form(BCR3) of the PML/RARα fusion genes are associated with early death in acute promylocytic leukemias.