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.
Han
Xin
X
Department of Hematopathology, The University of Texas M. D. Anderson Cancer Center, Houston 77030, USA.
Medeiros
L Jeffrey
LJ
Abruzzo
Lynne V
LV
Jones
Dan
D
Lin
Pei
P
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