TY - JOUR
T1 - Chronic myeloproliferative diseases with the t(5;12)(q33;p13)
T2 - Clonal evolution is associated with blast crisis
AU - Han, Xin
AU - Medeiros, L. Jeffrey
AU - Abruzzo, Lynne V.
AU - Jones, Dan
AU - Lin, Pei
PY - 2006/1
Y1 - 2006/1
N2 - 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.
AB - 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.
KW - Blast crisis
KW - Chronic myeloproliferative diseases
KW - Clonal evolution
KW - t(5;12)
UR - http://www.scopus.com/inward/record.url?scp=30644467488&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=30644467488&partnerID=8YFLogxK
U2 - 10.1309/Y3Y5G01443AD5L48
DO - 10.1309/Y3Y5G01443AD5L48
M3 - Article
C2 - 16482991
AN - SCOPUS:30644467488
SN - 0002-9173
VL - 125
SP - 49
EP - 56
JO - American journal of clinical pathology
JF - American journal of clinical pathology
IS - 1
ER -