Clinical characteristics and outcomes of mixed phenotype acute leukemia with Philadelphia chromosome positive and/or bcr-abl positive in adult.
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.
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.
Ying
Y
Wang
Min
M
Gu
Yingchang
Y
Mi
Lugui
L
Qiu
Shougeng
S
Bian
Jianxiang
J
Wang
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D005260:Female; D016044:Fusion Proteins, bcr-abl; D006801:Humans; D016130:Immunophenotyping; D053208:Kaplan-Meier Estimate; D007938:Leukemia / Q000235:genetics* / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010677:Philadelphia Chromosome; D011379:Prognosis; D016896:Treatment Outcome; D055815:Young Adult
D016044:Fusion Proteins, bcr-abl