| Literature DB >> 28369779 |
Francesco Onida1, Liesbeth C de Wreede2,3, Anja van Biezen2, Diderik-Jan Eikema2, Jenny L Byrne4, Anna P Iori5, Rik Schots6, Alexandra Jungova7, Johannes Schetelig8, Jürgen Finke9, Hendrik Veelken10, Jan-Erik Johansson11, Charles Craddock12, Matthias Stelljes13, Matthias Theobald14, Ernst Holler15, Urs Schanz16, Nicolaas Schaap17, Jörg Bittenbring18, Eduardo Olavarria19, Yves Chalandon20, Nicolaus Kröger21.
Abstract
Atypical chronic myeloid leukaemia (aCML) is an aggressive malignancy for which allogeneic haematopoietic stem cell transplantation (allo-HSCT) represents the only curative option. We describe transplant outcomes in 42 patients reported to the European Society for Blood and Marrow Transplantation (EBMT) registry who underwent allo-HSCT for aCML between 1997 and 2006. Median age was 46 years. Median time from diagnosis to transplant was 7 months. Disease status was first chronic phase in 69%. Donors were human leucocyte antigen (HLA)-identical siblings in 64% and matched unrelated (MUD) in 36%. A reduced intensity conditioning was employed in 24% of patients. T-cell depletion was applied in 87% and 26% of transplants from MUD and HLA-identical siblings, respectively. According to the EBMT risk-score, 45% of patients were 'low-risk', 31% 'intermediate-risk' and 24% 'high-risk'. Following allo-HSCT, 87% of patients achieved complete remission. At 5 years, relapse-free survival was 36% and non-relapse mortality (NRM) was 24%, while relapse occurred in 40%. Patient age and the EBMT score had an impact on overall survival. Relapse-free survival was higher in MUD than in HLA-identical sibling HSCT, with no difference in NRM. In conclusion, this study confirmed that allo-HSCT represents a valid strategy to achieve cure in a reasonable proportion of patients with aCML, with young patients with low EBMT risk score being the best candidates.Entities:
Keywords: Myelodyslastic/Myeloproliferative Neoplasms (MDS/MPN); Ph-negative CML: BCR-ABL1-negative; allogeneic transplantation; atypical chronic myeloid leukaemia
Mesh:
Year: 2017 PMID: 28369779 DOI: 10.1111/bjh.14619
Source DB: PubMed Journal: Br J Haematol ISSN: 0007-1048 Impact factor: 6.998