Adults With Acute Lymphoblastic Leukemia and Translocation (1;19) Abnormality Have a Favorable Outcome With Hyperfractionated Cyclophosphamide, Vincristine, Doxorubicin, and Dexamethasone Alternating With Methotrexate and High-Dose Cytarabine Chemotherapy

摘要: Chromosomal abnormalities in acute leukemia determine the disease pathophysiology and patient prognosis. Treatment of childhood lymphoblastic (ALL) results high cure rates 70% to 90%. Adults with ALL achieve complete remission 80% 90%; however, are only 30% 40%.1 A major difference between adult is frequency various cytogenetic subcategories. A hyperdiploid karyotype, which favorable, observed children compared 2% 5% adults. Another favorable category a translocation involving chromosomes 12 21, t(12;21), TEL-AML1 fusion gene.2 The latter most common molecular lesion but uncommon (range, 3%–4%).3,4 t(9;22), or Philadelphia chromosome (Ph) (an unfavorable subset), (25%) rare (<5%).5,6 One difficulty elucidating prognostic influence certain has been rarity disease7 coupled low specific abnormalities. One recurring both adults t(1;19)(q23;p13). It transcription factor 3 gene TCF3 (E2A) at 19p13 pre-B-cell homeobox PBX1 1q23, creating TCF3-PBX1 that encodes protein transforming properties. E2A 2 factors, E12 E47, which, turn, bind enhancer elements immunoglobulin κ IGK regulatory other genes. (HOX) on 1. E2A/PBX1 messenger RNAs formed code for chimeric proteins consist transcriptional activating domain E12/E47 DNA-binding domains PBX1.8,9 E2a/Pbx1 may promote leukemogenesis by transactivation several genes normally not expressed lymphoid tissues.10 t(1;19) occurs forms: 1) reciprocal t(1;19)(q23;p13) or, more often, 2) an unbalanced form characterized normal 1 19 rearranged 19, der(19)t(1,19)(q23;p13). In some studies, patients had worse outcome.11 Children who were treated conventional antimetabolite-based therapy protocols poor outcomes.12 Newer intensified regimens have improved prognosis, balanced still adverse prognosis.13 current study, we reviewed outcome received hyperfractionated cyclophosphamide, vincristine, doxorubicin (Adriamycin), dexamethasone alternating methotrexate high-dose cytarabine (hyper-CVAD) regimen.14