title: Safety and outcome of allogeneic stem cell transplantation in myelofibrosis.
abstract: OBJECTIVE
  We evaluated the safety and outcome of allo-HSCTs in myelofibrosis (MF).
  
  
  METHODS
  A total of 27 patients with primary (n = 20) or secondary (n = 7) MF, aged 51 (21-63) yr, transplanted from HLA-matched related (59%) or unrelated (41%) donors were analyzed. Conditioning was reduced in 26 and myeloablative in one patient; and ATG was used in 25. Sources of stem cells were as follows: peripheral blood (21), bone marrow (4) or both (2).
  
  
  RESULTS
  Prognostic factors that adversely affected overall survival (OS) in the multivariate analysis were as follows: recipient age >45 yr (HR = 10.55, P = 0.025) and unrelated donor (HR=3.73, P = 0.026). Post-transplant transfusion dependence adversely affected OS in the univariate analysis: dependence from either both RBCs and platelets (HR = 33.26, P = 0.001) or from either of them (HR = 10.53, P = 0.043). Of 16 JAK2V617F-positive patients evaluated post-transplant, it was eradicated in 69% and decreased in 25%. Acute GVHD III-IV developed in 19% and extensive chronic GVHD in 26% of patients; the relapse in four patients was treated with second allo-HSCT. Spleen decreased in all evaluated patients (n = 24). Fibrotic changes improved or disappeared in 80% of evaluated patients (n = 10).
  
  
  CONCLUSIONS
  Allo-HSCT may prolong survival, provide disease regression and improve quality of life in MF, especially in patients ≤ 45 yr transplanted from matched related donors. Achieving transfusion independence post-transplant indicates the favorable outcome.
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Miroslaw
authors.initials: M
authors.lastname: Markiewicz
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Monika
authors.initials: M
authors.lastname: Dzierzak Mietla
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Agata
authors.initials: A
authors.lastname: Wieczorkiewicz
authors.affiliation: Department of Public Health, Department of Organisation and Management, Faculty of Health Science, Wroclaw Medical University, Wroclaw, Poland.
authors.forename: Sylwia
authors.initials: S
authors.lastname: Mizia
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Grzegorz
authors.initials: G
authors.lastname: Helbig
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Malgorzata
authors.initials: M
authors.lastname: Kopera
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Bialas
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Malwina
authors.initials: M
authors.lastname: Rybicka
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Mariusz
authors.initials: M
authors.lastname: Matyja
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Koclega
authors.affiliation: School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Lech
authors.initials: L
authors.lastname: Sedlak
authors.affiliation: School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Tomasz
authors.initials: T
authors.lastname: Oleksy
authors.affiliation: School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Sundar
authors.initials: S
authors.lastname: Raman
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.
authors.forename: Slawomira
authors.initials: S
authors.lastname: Kyrcz-Krzemien
mesh_terms: D000328:Adult; D005260:Female; D006086:Graft vs Host Disease / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009154:Mutation; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000628:therapy*; D014019:Tissue Donors; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D053614:Janus Kinase 2
keywords: myelofibrosis; prognostic factors; transfusion; transplantation