title: Rapid regression of bone marrow fibrosis after dose-reduced allogeneic stem cell transplantation in patients with primary myelofibrosis.
abstract: OBJECTIVE
  To investigate the effect of a busulfan/fludarabine-based reduced intensity conditioning followed by allogeneic stem cell transplantation on regression of bone marrow fibrosis in patients with myelofibrosis.
  
  
  METHODS
  Twenty-four patients (male, n = 16; female, n = 8) with a median age of 52 years (range, 32-63 years) were included. Six patients were transplanted from human leukocyte antigen-identical siblings and 18 patients from matched unrelated donors. Diagnosis was primary myelofibrosis in 18 patients and secondary myelofibrosis in 6 patients; in 4 of them, primary myelofibrosis evolved from polycythemia vera, and in 2 of them from essential thrombocythemia. Using the European Consensus on grading bone marrow fibrosis, all patients had advanced marrow fibrosis MF-2 (n = 13) or MF-3 (n = 11) before allografting According to the Lille Risk Factor Scoring System, patients were classified as low risk (n = 5), intermediate risk (n = 16), or high risk (n = 3).
  
  
  RESULTS
  After stem cell transplantation, a complete (MF-0) or nearly complete (MF-1) regression of bone marrow fibrosis was seen in 59% at day +100, in 90% at day +180, and in 100% at day +360. No correlation between occurrence of acute graft-vs-host disease and fibrosis regression on day +180 was observed.
  
  
  CONCLUSIONS
  This study shows that allogeneic stem cell transplantation after reduced-intensity conditioning resulted in rapid regression of bone-marrow fibrosis.
authors.affiliation: Department of Stem Cell Transplantation, University Hospital Hamburg, Germany.
authors.forename: Nicolaus
authors.initials: N
authors.lastname: Kröger
authors.forename: Jürgen
authors.initials: J
authors.lastname: Thiele
authors.forename: Axel
authors.initials: A
authors.lastname: Zander
authors.forename: Rainer
authors.initials: R
authors.lastname: Schwerdtfeger
authors.forename: Guido
authors.initials: G
authors.lastname: Kobbe
authors.forename: Martin
authors.initials: M
authors.lastname: Bornhäuser
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Bethge
authors.forename: Jörg
authors.initials: J
authors.lastname: Schubert
authors.forename: Theo
authors.initials: T
authors.lastname: de Witte
authors.forename: Hans Michael
authors.initials: HM
authors.lastname: Kvasnicka
mesh_terms: D000328:Adult; D001856:Bone Marrow Examination; D002066:Busulfan / Q000008:administration & dosage; D005260:Female; D006086:Graft vs Host Disease; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D007700:Kinetics; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000628:therapy*; D012074:Remission Induction / Q000379:methods; D012720:Severity of Illness Index; D019172:Transplantation Conditioning / Q000379:methods; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D014740:Vidarabine / Q000008:administration & dosage / Q000031:analogs & derivatives
chemical_list: D014740:Vidarabine; D002066:Busulfan; C024352:fludarabine