[The role of BCR/ABL isoforms in the presentations and outcome of Philadelphia-positive acute lymphoblastic leukemia in adult patients].
OBJECTIVE
To investigate the difference of clinical characteristics and outcomes between different isoforms of BCR/ABL in adults with Philadelphia-positive acute lymphoblastic leukemia (ALL).


METHODS
The data of 106 adults with Ph+ ALL diagnosed in our hospital from January 1, 1996 to December 31, 2007 were reviewed. The difference of clinical characteristics between different subgroups of BCR/ABL was compared and their relation with outcomes was studied.


RESULTS
The median age of the 106 patients was 34 years and the median white blood cell count at baseline was 28.5 x 10(9)/L. Comparative analysis demonstrated that patients in p210 group had an older age, higher blood platelet count (BPC) and more frequent occurrence of splenomegaly. Referring to the outcomes, the complete remission (CR) rate of the two groups were 92.2% and 93.9%, respectively. The median overall survival (OS) and relapse free survival (RFS) in p190 group were 13 months and 10 months, the 1, 3-year estimated OS were (54.7 +/- 6.7)% and (5.5 +/- 5.2)%, and the 1, 3-year estimated RFS were (40.2 +/- 6.8)% and (7.8 +/- 6.7)%, while in p210 group, the median OS and RFS were 15 months and 10 months, respectively, the 1, 3-year estimated OS were (65.8 +/- 8.9)% and (14.5 +/- 7.4)%, and the 1, 3-year estimated RFS were (48.3 +/- 9.4)% and (12.9 +/- 7.7)%. All of the above data had no statistic significance between the two groups.


CONCLUSIONS
Majority of the adults with Ph+ ALL is p190 positive and patients with p210 have older age, higher BPC and more frequent occurrence of splenomegaly, while there is no significant difference between p190 group and p210 group in CR rate, RFS and OS.
Li
Ye-Nan
YN
Institute of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin, China.
Zou
De-Hui
DH
Gu
Min
M
Mi
Ying-Chang
YC
Wang
Jian-Xiang
JX
Qiu
Lu-Gui
LG
D000293:Adolescent; D000328:Adult; D005260:Female; D016044:Fusion Proteins, bcr-abl / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010677:Philadelphia Chromosome; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000145:classification / Q000175:diagnosis* / Q000235:genetics*; D011379:Prognosis; D020033:Protein Isoforms / Q000235:genetics; D012189:Retrospective Studies; D055815:Young Adult
D020033:Protein Isoforms; C406060:abl-bcr fusion protein, human; D016044:Fusion Proteins, bcr-abl