Children with hyperdiploid but not triple trisomy (+4, +10, +17) acute lymphoblastic leukemia have an increased incidence of extramedullar relapse on current therapies: A single institution experience

Department of Pediatrics and Communicable Diseases, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan, USA.
American Journal of Hematology (Impact Factor: 3.8). 01/2008; 83(1):34-40. DOI: 10.1002/ajh.21011
Source: PubMed


To evaluate the outcome of children with high hyperdiploid acute lymphoblastic leukemia (hHDALL) treated at the author's institution. One hundred thirty-five consecutive children with B-precursor ALL were diagnosed between 1991 and 2002: 38 (28.1%) hHDALL and 97 (71.9%) non-hHDALL. In the hHDALL group, 11/38 (28.9%) relapsed at a median interval of 2.8 years (range: 0.8-5.0 years) with 9/11 relapses occurring at the end or after the completion of therapy. Three (27.3%) relapses were isolated hematopoietic (BM), while eight (72.7%) were either isolated extramedullary (EM) relapses (n=6; Testis: 4; CNS: 2) or combined hematopoietic and extramedullary relapses (n=2; BM + CNS: 1; BM + Testis: 1). For the non-hHDALL group, 29/97 (29.9%) relapsed. Unlike the hHDALL group, the non-hHDALL group experienced hematopoietic relapses (62%; n=18) more frequently than isolated extramedullary (27.5%; n=8: Testis: 1; CNS: 7) or combined hematopoietic and extramedullary relapses (10.3%; CNS + BM: 3), with 24/29 (82.8%) of the relapses occurring on therapy. Relapses in hHDALL frequently involved EM sites (P=0.053). Presence of triple trisomy of +4,+10,+17 at diagnosis had a protective effect against relapse (P<0.05). Five-year EFS for the hHDALL and non-hHDALL patients was similar, 70.5+/-7.5% and 66.4+/-4.9%, respectively. Five-year OS for the hHDALL patients was significantly higher than for the non-hHDALL patients, 92+/-4.5% vs. 74.1+/-4.5%, P=0.038. Biologically significant differences exist between relapse patterns of hHDALL and non-hHDALL cases related to relapse sites and time periods when relapses occur. hHDALL relapses continue to be chemo-sensitive.

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Available from: Jeffrey W Taub, Oct 02, 2015
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    • "Giemsa banded karyotypes were analyzed by one of us (LK) and the chromosomes were counted and identified using standard International System for Cytogenetic nomenclature 2005 method.[6] FLT3 internal tandem duplication and tyrosine kinase domain (ITD and TKD) mutations were tested using standard techniques which were already applied in this laboratory.[7] "
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