Clinical and laboratorial analysis for 15 adult cases of mixed phenotypic acute leukemia with Ph chromosome and/or positive BCR-ABL.
10.7534/j.issn.1009-2137.2013.05.006
- Author:
Ling-Zhi YAN
1
;
Su-Ning CHEN
;
Na-Na PING
;
Qin-Rong WANG
;
Hong LIU
;
Zi-Xuan DING
;
Ming-Qing ZHU
;
Jian-Ying LIANG
;
Dan-Dan LIU
;
Jian-Nong CEN
;
Jin-Lan PAN
;
Hui-Ying QIU
;
Ai-Ning SUN
;
De-Pei WU
Author Information
1. The First Affiliated Hospital of Soochow University, Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, Jiangsu Province, China.
- Publication Type:Journal Article
- MeSH:
Adolescent;
Adult;
Aged;
Aged, 80 and over;
Antigens, CD34;
metabolism;
Female;
Fusion Proteins, bcr-abl;
genetics;
metabolism;
Hematopoietic Stem Cell Transplantation;
Humans;
Karyotyping;
Male;
Middle Aged;
Phenotype;
Precursor Cell Lymphoblastic Leukemia-Lymphoma;
diagnosis;
genetics;
therapy;
Prognosis;
Protein Kinase Inhibitors;
therapeutic use;
Retrospective Studies;
Survival Rate;
Young Adult
- From:
Journal of Experimental Hematology
2013;21(5):1116-1120
- CountryChina
- Language:Chinese
-
Abstract:
The purpose of this study was to summary the clinical and laboratorial features in 15 adult cases of mixed phenotypic acute leukemia with Ph chromosome and/or BCR-ABL fusion gene positive (Ph(+)MPAL), 15 adult patients with Ph(+)MPAL were defined by WHO-2008 classification. The clinical characteristics, results of morphology, immunology, cytogenetics and molecular genetic detections and results of follow-up in 15 adult patients with Ph(+)MPAL were analyzed retrospectively. The results showed that 15 patients among 87 cases of MPAL demonstrated Ph(+)MPAL (17.2%; 15/87) (7 males and 8 females), their median age was 51 (range 16-81) year old and median WBC count at diagnosis was 69 (12.7-921)×10(9)/L. Based on FAB criteria, these patients showed different morphologic types, including AML (13.3%; 2/15), ALL (40.0%; 6/15), HAL (46.7%; 7/15). Immunologic analysis indicated that 15 cases of Ph(-)MPAL were all classified as B-lymphoid +myeloid mixed immunophenotype. Except one patient, all expressed CD34 antigen on the surface of leukemia cells with 64.3% strong positive, only Ph (53.3%; 8/15), Ph with additional chromosomal abnormalities (33.3%; 5/15) and normal karyotype (13.3%; 2/15) were cytogenetically identified. BCR-ABL fusion gene transcript positive were detected by multiplex reverse transcription PCR in all cases, with e1a2 subtype (p190) (40.0%; 6/15) and b2a2 or b3a2 (p210) subtype (60.0%; 9/15). Four out of 7 (57.1%) patients were found to have IKZF1 gene deletion, without other common gene mutations. Seven out of 10 cases (70.0%) achieved complete remission (CR) after one cycle of induction chemotherapy. In the induction stage, CR rate seemed higher when tyrosine kinase inhibitors (TKI) were added to chemotherapy (83.3%:50.0%; P = 0.206). Overall survival (OS) in 4 patients received allogeneic hematopoietic stem cell transplantation (allo-HSCT) was longer than that in 4 patients received chemotherapy alone (P = 0.004). It is concluded that Ph(+)MPAL mainly is expressed as B+My phenotype. The majority of patients is older and has CD34 overexpression. In the aspect of molecular genetics, the Ph(+)MPAL is similar to other acute leukemia with Ph chromosome. Ph(+)MPAL is a subtype of acute leukemia with poor prognosis. WBC count at diagnosis is an independent prognostic factor. The combination of TKI and allo-HSCT can improve their long-term survival, which needs to be confirmed through carrying out a prospective and multicenter clinical trial for newly diagnosed Ph(+)MPAL.
