Authors: Bin Fu Chi Young Ok Maitrayee Goswami Wei Xei Jesse M Jaso Tariq Muzzafar Carlos BuesoRamos Srdan Verstovsek Guillermo GarciaManero L Jeffrey Medeiros Sa A Wang
Publish Date: 2013/05/10
Volume: 92, Issue: 10, Pages: 1335-1343
Abstract
The presence of moderate to severe bone marrow BM fibrosis has been shown to be an adverse feature in patients with primary myelodysplastic syndromes MDS However the clinical importance of BM fibrosis is not clear in therapyrelated MDS We retrieved all therapyrelated MDS tMDS cases n = 266 diagnosed at our hospital over a 10year period 2003–2012 Reticulin and trichrome stains were performed in cases in which BM fibrosis was suspected on initial evaluation of hematoxylin and eosinstained slide BM fibrosis was graded according to European consensus guidelines and a score of MF2/MF3 was defined as moderate/severe fibrosis Moderate/severe BM fibrosis was found in 47 17 patients Compared to 219 patients with no/mild BM fibrosis the patients with moderate/severe fibrosis presented with severer thrombocytopenia p = 0039 and higher numbers of circulating blasts p = 0051 but with similar degrees of anemia and neutropenia transfusion requirements and similar incidences of hepatosplenomegaly and constitutional symptoms Histological examination revealed a comparable BM cellularity and BM blast percentage but markedly increased megakaryocytes p 0001 in the fibrotic group Although the risk distribution of cytogenetic data was similar according to the New Comprehensive Cytogenetic Scoring criteria −5 and −17 were more frequently observed in tMDS with moderate/severe BM fibrosis p = 0031 and p = 0043 respectively With a median followup of 115 months patients with moderate/severe BM fibrosis showed a similar risk of acute myeloid leukemia transformation and a comparable overall survival in univariate and multivariate analyses Moderate/severe BM fibrosis in patients with tMDS is associated with certain clinicopathological and genetic features However unlike the situation in patients with primary MDS moderate/severe BM fibrosis does not add additional risk to patients with therapyrelated MDS
Keywords: