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Mixed phenotype acute leukemia: a study of 61 cases using world health organization and European group for the immunological classification of leukaemias criteria.

混合型急性白血病:采用世界卫生组织和欧洲白血病标准进行免疫学分类的61例研究。

Weinberg OK,Seetharam M,Ren L,Alizadeh A,Arber DA

Abstract

The 2008 World Health Organization (WHO) classification system grouped bilineal and biphenotypic acute leukemias together under a new heading of mixed phenotype acute leukemia (MPAL). The lineage-specific marker criteria have also changed for a diagnosis of MPAL. The goal of this study was to characterize clinical significance of this new group.
Sixty-one patients diagnosed with MPAL using either European Group for the Immunological Classification of Leukaemias (EGIL) criteria or 2008 WHO criteria were included in this study.
Sixteen patients (26%) diagnosed with acute biphenotypic leukemia using EGIL criteria did not fulfill 2008 WHO criteria for MPAL. Cytogenetic data were available for 32 patients, and the most common abnormality was t(9;22) (five of 32 cases). Clinical outcome data suggested that younger patients with MPAL (≤21 years) had better overall survival (OS) in both the EGIL and WHO groups (EGIL, P = .0403; WHO, P = .0601). Compared with 177 patients with acute myeloid leukemia (AML), MPAL patients had better OS (P = .0003) and progression-free survival (P = .0001). However, no difference in OS between MPAL and 387 patients with acute lymphoblastic leukemia was present (P = .599).
As defined by the 2008 WHO classification, fewer patients are now classified as having MPAL than with the EGIL criteria. In this study, patients with MPAL have a better clinical outcome compared with patients with AML.

摘要

2008年世界卫生组织(WHO)分类系统把双系双表型急性白血病归入混合型急性白血病(MPAL)这一新类型下。对诊断MPAL的谱系特异性标记的标准也发生改变。本研究的目的是为了确定这种新类型的临床意义。
这项研究纳入了欧洲白血病免疫学分类(EGIL)标准或2008年WHO标准确诊为MPAL的61例患者。
16例(26%)用EGIL标准诊断的急性双表型白血病却不符合2008年MPAL的WHO标准。32例有可用的细胞遗传学数据,其中最常见的异常基因是t(9;22)(5/32例)。临床结果的数据表明,同时符合EGIL和世界卫生组织标准的年轻MPAL患者(≤21岁)有着更长的总体生存期(OS)(EGIL,P =0.0403;世界卫生组织,P =0.0601)。与177例急性髓系白血病(AML)相比,MPAL患者有更长的OS(P=0.0003)和无进展生存期(P =0.0001)。然而,MPAL和387例急性淋巴细胞白血病患者的OS却没有差异(P=0.599)。
按2008年世界卫生组织的分类定义,归类为MPAL的患者比按EGIL标准更少。在这项研究中,与AML患者相比,MPAL患者有更好的临床结果。

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