混合表型急性白血病EGIL1998诊断积分系统和WHO2008诊断分型标准比较  

Comparison of EGIL1998 and WHO2008 criteria for the diagnosis of mixed phenotype acute leukemia

作　　者：叶蕾 林冬 秘营昌 张静[1] 王迎 王慧君 魏辉 刘兵城 周春林 李巍 王建祥 

机构地区：[1]中国医学科学院、北京协和医学院血液学研究所、血液病医院,天津300020

出　　处：《中华血液学杂志》2012年第4期286-290,共5页Chinese Journal of Hematology

基　　金：国家科技支撑计划课题（2008BA161801）;国家科技重大专项课题（2008ZX09312-026、2011ZX09302-007-014）

摘　　要：目的分析混合表型急性白血病（MPAL）EGIL1998诊断积分系统和WH02008诊断分型标准在临床应用中的关系。方法分别按EGIL1998诊断积分系统和WH02008诊断分型标准诊断MPAL，比较两种标准的诊断符合率和不同标准诊断组患者治疗反应。结果①1996年2月至2010年10月共收治原发初治急性白血病（AL）患者1835例。按EGIL1998标准诊断为MPAL的患者共74例，占AL的4．O％；以M／B混合表型最多见（54例，占73．0％）。按WH02008标准诊断为MPAL81例，占AL的4．4％；也以M／B混合表型最多见（63例，占77．8％）。EGIL1998和WH02008标准诊断MPAL的诊断符合率为79．7％（74例中59例），M／B、M／T、M／B／T、B／T混合表型AL的诊断符合率分别为85．2％（54例中46例）、56．3％（16例中9例）、0（2例中0例）和50．0％（2例中1例）。按EGIL1998标准不符合MPAL诊断的1761例患者，经WH02008标准复核新增22例MPAL。②经WH02008标准诊断为MPAL的患者中，包括伴特异染色体异常t（9；22）（q34；q11．2）／BCR-ABL1者13例，t（v；11q23）／MLL重排者1例。M／B—MPAL非特指型49例，M／T-MPAL非特指型14例，MPAL非特指罕见类型4例。③符合EGIL1998标准、符合WH02008标准、符合两种标准、只符合EGIL1998标准和只符合WH02008标准MPAL患者完全缓解率分别为69．0％、73．5％、73．5％、44．4％、73．7％，组间差异无统计学意义（P值均〉0．05）。结论EGIL1998诊断积分系统和WH02008诊断分型标准诊断MPAL具有交叉、互补性。Objective To explore the relationship between EGIL 1998 and WHO2008 criteria for the diagnosis of mixed phenotype acute leukemia （MPAL）. Methods The same group patients were diagnosed for MPAL by EGIL 1998 and WHO2008 criteria, respectively. The coincidence rate of diagnosis and therapeutic response of two diagnosis standards were compared. Results A cohort of 1835 de novo acute leukemia （AL） patients admitted to our hospital from February 1996 to October 2010 were retrospectively analyzed by applying both EGIL1998 and WHO2008 classification criteria. Seventy four patients were diagnosed with MPAL according to EGIL 1998, accounting for 4.0% of all AL cases. The main subtype is M/B （54 cases, 73.0% ）. While 81 patients were diagnosed as MPAL, accounting for 4.4% based on WHO 2008 criteria. The most common type is also M/B （63 cases, 77.8% ）. Fifty nine （79.7%） cases met both criteria. In the subtypes of M/B, M/T, M/B/T and B/T, the coincidence rate is 85.2% （46/54） , 56.3% （9/16） , 0（0/2） and 50.0% （1/2） , respectively. In the 1761 cases excluded as MPAL by EGIL1998, 22 cases can be diagnosed as MPAL by WHO 2008 classification. （2）Among the patients diagnosed as B/M MPAL by WHO2008, 13 were MPAL with t （9; 22） （q34; qll. 2）/BCR-ABL1, 1 was MPAL with t （v; 11q23）/ MLL-rearranged, 49 was B/myeloid not otherwise specified （NOS） , 14 was T/myeloid NOS and 4 was MPAL NOS-rare types. （3）The overall final complete remission rate of the patients diagnosed by EGIL1998, 2008WHO, met both criteria, EGIL1998 but excluded by 2008 WHO and by 2008 WHO but excluded by EGIL1998 was 69.0% , 73.5%, 73.5% , 44.4% and 73.7%, respectively, with no significant difference between any two groups based on X2 test （P 〉 0.05 ）. Conclusions EGIL 1998 and WHO2008 criteria have reciprocity in the diagnosis of MPAL.

关 键 词：白血病 混合细胞 急性 EGIL1998诊断积分系统 WHO2008诊断分型标准 

分 类 号：R733.71[医药卫生—肿瘤]