三种 PML／RARA 亚型急性早幼粒细胞白血病临床特点对比分析

摘要

Objective To analyze and compare the clinical characteristics of acute promyelocytic leukemia ( APL) with promyelocytic leukemia ( PML)/retinoic acid receptorαgene ( RARA) different isoforms.Methods The clinical data from 108 APL patients in our center from 2010 to 2014 were collected and the PML/RARA different isoforms were ana-lyzed.Results ①The most common PML/RARA subtype was L isoform, which were 67 cases (62.0%), followed by 30 cases of S (27.8%) and 11 cases of V (10.2%) .②The bleeding was less frequently found in V isoform (45.5%) than in L isoform (74.6%) and S isoform (86.7%) (P=0.026).The median fibrinogen level in V isoform (1.98 g/L) was higher than that in L isoform (1.18 g/L) and S isoform (1.05 g/L) (P=0.024).③The prevalence of FLT3/ITD muta-tion was more frequently found in S isoform (26.7%) than in L isoform (7.6%) and V isoform (0%) (P=0.013).The positive rate of CD34 expression was higher in S isoform (24.1%) than in L isoform (6.3%) and V isoform (11.1%) (P=0.046).④The incidences of retinoic acid syndrome in the high risk group, medium risk group and low risk group were 56.3%, 28.8%and 17.5%, respectively (P=0.021).The difference of the incidence of retinoic acid syndrome in three PML/RARA isoforms was not significant (P=0.643).Conclusions The clinical characteristics of three PML/RARA iso-forms were not exactly the same.The most common subtype was L isoform, the lowest incidence of bleeding was found in V isoform, and S isoform was most likely to be accompanied by FLT3/ITD mutation and CD34 expression.%目的：分析并比较不同早幼粒细胞白血病／维甲酸受体α融合基因（ PML／RARA）亚型急性早幼粒细胞白血病（ APL）的临床特点。方法收集我中心2010～2014年108例初诊APL患者的临床资料，分析不同PML／RARA亚型的特点。结果最常见的PML／RARA亚型为L型（67例，62．0％），其次为S型（30例，27．8％）、V型（11例，10．2％）。 V型患者合并出血的比例（45．5％）低于L型（74．6％）和S型（86．7％），P均＜0．05；V型患者中位纤维蛋白原水平（1．98 g／L）高于L型（1．18 g／L）和S型（1．05 g／L），P均＜0．05。 S型患者（26．7％）较L型（7．6％）和V型（0）更易伴发FMS样酪氨酸酶3内部串联重复（FLT3／ITD）突变，P均＜0．05。 S型患者CD34表达阳性率（24．1％）高于L型（6．3％）和V型（11．1％），P均＜0．05。高、中、低危组维甲酸综合征发生率分别为56．3％、28．8％、17．5％（ P均＜0．05）。三种PML／RARA亚型维甲酸综合征的发生率差异无统计学意义。结论三种PML／RARA亚型的APL临床特点不尽相同，L型最常见，V型出血发生率较低，S型较易伴FLT3／ITD突变和CD34阳性表达。