Leukemia Diagnosis and Treatment Center, State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, 288 Nanjing Road, Tianjin 300020, China.
Int J Hematol. 2011 Dec;94(6):552-5. doi: 10.1007/s12185-011-0953-1. Epub 2011 Oct 21.
Knowledge of mixed phenotype acute leukemia (MPAL) with t(9;22)(q34;q11.2) and/or bcr-abl (Ph-positive MPAL) is limited. In this report, we review 21 adult patients with Ph-positive and/or bcr-abl positive MPAL. They were predominantly male, and presented with high WBC counts; 61.9% patients had WBC counts higher than 30 × 10(9)/L, and 33.3% patients had WBC counts higher than 100 × 10(9)/L. Electron microscopy (EM)-determined positivity for myeloperoxidase (MPO) should be considered for the classification of acute leukemia because MPO was positive by EM and flow cytometry only in 14.3% of cases in our study. Six cases (30.0%) had additional chromosome aberrations. Expression of p190(bcr-abl) was more common than that of p210(bcr-abl). There was no difference in characteristics between the p190(bcr-abl) positive and p210(bcr-abl) positive groups. The overall complete remission (CR) rate was 81.0%. Females, and patients with high WBC (>100 × 10(9)/L) at baseline had lower CR rate (57.1, 57.1%, respectively). The treatment outcome of Ph-positive MPAL is poor; the 1-year overall survival (OS) and relapse-free survival (RFS) rate were 28.0 and 18.0%, respectively. Imatinib and allogeneic hematopoietic stem cell transplantation can improve survival with Ph-positive MPAL.
混合表型急性白血病（MPAL）伴 t（9;22）（q34;q11.2）和/或 bcr-abl（Ph 阳性 MPAL）的相关知识有限。本研究回顾了 21 例成人 Ph 阳性和/或 bcr-abl 阳性 MPAL 患者。这些患者主要为男性，表现为白细胞计数较高；61.9%的患者白细胞计数高于 30×10(9)/L，33.3%的患者白细胞计数高于 100×10(9)/L。电子显微镜（EM）确定的髓过氧化物酶（MPO）阳性应考虑用于急性白血病的分类，因为在本研究中，只有 14.3%的病例 EM 和流式细胞术检查 MPO 阳性。6 例（30.0%）存在额外的染色体异常。p190(bcr-abl)的表达比 p210(bcr-abl)更常见。p190(bcr-abl)阳性组和 p210(bcr-abl)阳性组的特征无差异。总体完全缓解（CR）率为 81.0%。女性和基线时白细胞计数较高（>100×10(9)/L）的患者 CR 率较低（分别为 57.1%、57.1%）。Ph 阳性 MPAL 的治疗效果较差；1 年总生存率（OS）和无复发生存率（RFS）分别为 28.0%和 18.0%。伊马替尼和异基因造血干细胞移植可改善 Ph 阳性 MPAL 的生存。
