Erschienen in:
01.12.2011 | Case Report
Clinical characteristics and outcomes of mixed phenotype acute leukemia with Philadelphia chromosome positive and/or bcr-abl positive in adult
verfasst von:
Ying Wang, Min Gu, Yingchang Mi, Lugui Qiu, Shougeng Bian, Jianxiang Wang
Erschienen in:
International Journal of Hematology
|
Ausgabe 6/2011
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Abstract
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 × 109/L, and 33.3% patients had WBC counts higher than 100 × 109/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 × 109/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.