Chronic myeloproliferative diseases with the t(5;12)(q33;p13): clonal evolution is associated with blast crisis.
Chronic myeloproliferative diseases (CMPDs) associated with t(5;12)(q33;p13) are reported to be responsive to imatinib mesylate. We studied 5 cases of CMPD with isolated t(5;12) treated at our hospital between January 1993 and October 2004. All were men with a median age of 55 years (range, 18-68 years). In the peripheral blood, each had marked leukocytosis, with variable eosinophilia (n = 4), monocytosis (n = 3), or basophilia (n = 2). Bone marrow specimens were hypercellular (70%-100%) with marked myeloid hyperplasia in each patient, but only 1 patient had eosinophilia, monocytosis, and basophilia. Follow-up ranged from 23 to 182 months (median, 48 months). Four died 23 to 182 months after initial diagnosis, 3 of blast crisis and 1 of cardiac complications of severe eosinophilia. Additional cytogenetic aberrations were identified at the time of blast crisis. Of 3 patients treated with imatinib, 2 responded, but only 1 had a sustained response. CMPD with t(5;12) commonly transforms to blast phase, and transformation is associated with cytogenetic evidence of clonal evolution.
Department of Hematopathology, The University of Texas M. D. Anderson Cancer Center, Houston 77030, USA.
Xin
X
Han
L Jeffrey
LJ
Medeiros
Lynne V
LV
Abruzzo
Dan
D
Jones
Pei
P
Lin
D000293:Adolescent; D000328:Adult; D000368:Aged; D001549:Benzamides; D001752:Blast Crisis / Q000235:genetics / Q000473:pathology*; D001854:Bone Marrow Cells / Q000473:pathology; D002869:Chromosome Aberrations; D002881:Chromosomes, Human, Pair 12; D002895:Chromosomes, Human, Pair 5; D002999:Clone Cells / Q000473:pathology*; D018450:Disease Progression; D006801:Humans; D000068877:Imatinib Mesylate; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D010879:Piperazines / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use
D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate