title: Deletion of any part of the BCR or ABL gene on the derivative chromosome 9 is a poor prognostic marker in chronic myelogenous leukemia.
abstract: To evaluate the prognostic significance of submicroscopic deletions of the ABL or BCR gene associated with t(9;22) in chronic myelogenous leukemia (CML), we investigated the incidence of an ABL or BCR deletion on derivative chromosome 9 using fluorescence in situ hybridization (FISH). FISH was performed using the LSI BCR/ABL dual-fusion translocation probe on bone marrow cells of 86 patients with CML. Of 86 patients, ABL deletion was detected in 13 (15.1%) patients and BCR deletion in 8 patients (9.3%). Patients with ABL deletion showed shorter event-free survival time (EFS) than those without ABL deletion (P = 0.020). Patients with BCR deletion showed significantly short overall survival time (OS; P = 0.039). Patients with ABL and/or BCR deletion (14/86 patients, 16.3%) showed significantly short OS and EFS (median OS, 43.0 months; median EFS, 40.0 months), compared to the patients without any BCR or ABL gene deletions (median OS, 94.0 months; median EFS, 90.0 months; P = 0.041 for OS, P = 0.008 for EFS). All the patients with BCR deletion, except for one, had a concomitant ABL deletion, suggesting that BCR deletion occurs in conjunction with ABL deletion. In patients with ABL deletion only, BCR/ABL rearrangement with b2a2 mRNA type tended to be more frequent than in patients without any deletion of the two genes (P = 0.073). Deletion of any of the BCR or ABL genes on derivative chromosome 9 was associated with both short OS and EFS. We conclude that deletion of not only the ABL gene, but also of the BCR gene, is a poor prognostic marker that indicates rapid disease progression in CML.
authors.affiliation: Department of Laboratory Medicine, Hallym University College of Medicine, 39 Hallymdaehak-gil, Chuncheon, 200-702, Seoul, Korea.
authors.forename: Young Kyung
authors.initials: YK
authors.lastname: Lee
authors.forename: Young Ree
authors.initials: YR
authors.lastname: Kim
authors.forename: Hyun Chung
authors.initials: HC
authors.lastname: Min
authors.forename: Bo Ra
authors.initials: BR
authors.lastname: Oh
authors.forename: Tae Young
authors.initials: TY
authors.lastname: Kim
authors.forename: Young Soo
authors.initials: YS
authors.lastname: Kim
authors.forename: Han Ik
authors.initials: HI
authors.lastname: Cho
authors.forename: Hee Chan
authors.initials: HC
authors.lastname: Kim
authors.forename: Yun Song
authors.initials: YS
authors.lastname: Lee
authors.forename: Dong Soon
authors.initials: DS
authors.lastname: Lee
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000737:chemistry / Q000378:metabolism / Q000473:pathology; D002648:Child; D002899:Chromosomes, Human, Pair 9 / Q000235:genetics*; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D016044:Fusion Proteins, bcr-abl / Q000235:genetics; D017353:Gene Deletion; D016313:Genes, abl / Q000235:genetics*; D006801:Humans; D017404:In Situ Hybridization, Fluorescence / Q000379:methods*; D015994:Incidence; D007723:Korea / Q000453:epidemiology; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000175:diagnosis / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D051562:Proto-Oncogene Proteins c-bcr / Q000235:genetics*; D015996:Survival Rate
chemical_list: D016044:Fusion Proteins, bcr-abl; C496613:BCR protein, human; D051562:Proto-Oncogene Proteins c-bcr