title: The clinical importance of moderate/severe bone marrow fibrosis in patients with therapy-related myelodysplastic syndromes.
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 therapy-related MDS. We retrieved all therapy-related MDS (t-MDS) cases (n = 266) diagnosed at our hospital over a 10-year period (2003-2012). Reticulin and trichrome stains were performed in cases in which BM fibrosis was suspected on initial evaluation of hematoxylin and eosin-stained 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 = 0.039) and higher numbers of circulating blasts (p = 0.051) 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 < 0.001) 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 t-MDS with moderate/severe BM fibrosis (p = 0.031 and p = 0.043, respectively). With a median follow-up of 11.5 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 t-MDS 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 therapy-related MDS.
authors: Department of Hematopathology, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Blvd., Unit 72, Houston, TX 77030, USA.
authors: Bin
authors: B
authors: Fu
authors: Chi Young
authors: CY
authors: Ok
authors: Maitrayee
authors: M
authors: Goswami
authors: Wei
authors: W
authors: Xei
authors: Jesse M
authors: JM
authors: Jaso
authors: Tariq
authors: T
authors: Muzzafar
authors: Carlos
authors: C
authors: Bueso-Ramos
authors: Srdan
authors: S
authors: Verstovsek
authors: Guillermo
authors: G
authors: Garcia-Manero
authors: L Jeffrey
authors: LJ
authors: Medeiros
authors: Sa A
authors: SA
authors: Wang
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009190:Myelodysplastic Syndromes / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology*; D009687:Nuclear Proteins / Q000235:genetics; D000090243:Nucleophosmin; D055728:Primary Myelofibrosis / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D012189:Retrospective Studies; D055815:Young Adult; D051941:fms-Like Tyrosine Kinase 3 / Q000235:genetics
chemical_list: D009687:Nuclear Proteins; D000090243:Nucleophosmin; C497970:FLT3 protein, human; D019009:Proto-Oncogene Proteins c-kit; D051941:fms-Like Tyrosine Kinase 3; C507924:JAK2 protein, human; D053614:Janus Kinase 2
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