title: Bone marrow fibrosis in patients with primary myelodysplastic syndromes has prognostic value using current therapies and new risk stratification systems.
abstract: Bone marrow fibrosis has recently been recognized as an adverse histological feature in patients with primary myelodysplastic syndromes. In this study, we assessed the prognostic impact of bone marrow fibrosis in patients with primary myelodysplastic syndromes under the recently revised new risk stratification systems: the New Comprehensive Cytogenetic Scoring System and the Revised International Prognostic Scoring System. From 2002 to 2012, a total of 79 (13%) patients with primary myelodysplastic syndromes and moderate/severe bone marrow fibrosis were identified; and these patients were compared with a control group of 166 patients with myelodysplastic syndromes but no significant fibrosis. Bone marrow fibrosis predicted an inferior overall survival and leukemia event-free survival for patients who received no hematopoietic stem cell transplant in univariate and multivariate analysis. Eleven patients with bone marrow fibrosis and 32 control group patients underwent hematopoietic stem cell transplant; and bone marrow fibrosis was an independent risk for an inferior overall survival but not leukemia-free survival. In addition, 17 (4%) patients developed bone marrow fibrosis during the course of myelodysplastic syndromes, which was accompanied by clinical and cytogenetic evidence of disease progression. JAK2 V617F mutations were detected in 6 of the 28 patients with bone marrow fibrosis presenting at the time of diagnosis and 2 of the 7 patients with bone marrow fibrosis developing in the course of disease, significantly higher than the control group patients. We conclude that bone marrow fibrosis is an adverse risk feature in primary myelodysplastic syndromes in the current therapeutic era, and this risk feature is not captured by newly revised risk stratification systems. Inclusion of bone marrow fibrosis in patient assessment may further aid in risk-adapted therapeutic decisions.
authors.affiliation: 1] Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA [2] Department of Hematology, Central South University Xiangya Hospital, Changsha, China.
authors.forename: Bin
authors.initials: B
authors.lastname: Fu
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Jesse M
authors.initials: JM
authors.lastname: Jaso
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Rachel L
authors.initials: RL
authors.lastname: Sargent
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Maitrayee
authors.initials: M
authors.lastname: Goswami
authors.affiliation: Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Srdan
authors.initials: S
authors.lastname: Verstovsek
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Sa A
authors.initials: SA
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001853:Bone Marrow / Q000473:pathology*; D001856:Bone Marrow Examination; D018450:Disease Progression; D005260:Female; D005355:Fibrosis; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009190:Myelodysplastic Syndromes / Q000235:genetics / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate; D055815:Young Adult
chemical_list: D053614:Janus Kinase 2