title: Syngeneic stem cell transplant for spent-phase polycythaemia vera: eradication of myelofibrosis and restoration of normal haematopoiesis.
abstract: We report a patient with spent-phase polycythaemia vera (S-PV) and massive splenomegaly who failed to engraft after a syngeneic granulocyte colony-stimulating factor-primed peripheral blood stem cell transplant (SCT), but later engrafted after splenectomy. Bone marrow (BM) showed resolution of myelofibrosis (MF) and absent endogenous erythroid colonies. This case demonstrated that (1) normal haematopoiesis can be restored after syngeneic SCT despite extensive MF, and (2) fibrosis can regress following a total body irradiation-containing regimen and syngeneic SCT. As a graft-versus-BM stroma effect is non-existent in syngeneic transplants, there may be a role for autologous SCT to obliterate MF in S-PV.
authors.affiliation: Division of Hematology, Department of Medicine, Mount Sinai Medical Center, New York, NY 10029, USA.
authors.forename: Shambavi
authors.initials: S
authors.lastname: Richard
authors.forename: Luis
authors.initials: L
authors.lastname: Isola
authors.forename: Eileen
authors.initials: E
authors.lastname: Scigliano
authors.forename: Harvinder
authors.initials: H
authors.lastname: Singh
authors.forename: Vesna
authors.initials: V
authors.lastname: Najfeld
authors.forename: Harriet
authors.initials: H
authors.lastname: Gilbert
authors.forename: Rona S
authors.initials: RS
authors.lastname: Weinberg
authors.forename: Steven
authors.initials: S
authors.lastname: Fruchtman
mesh_terms: D003131:Combined Modality Therapy; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D008875:Middle Aged; D011087:Polycythemia Vera / Q000628:therapy*; D055728:Primary Myelofibrosis / Q000628:therapy; D013156:Splenectomy; D014185:Transplantation, Isogeneic; D014916:Whole-Body Irradiation