Syngeneic stem cell transplant for spent-phase polycythaemia vera: eradication of myelofibrosis and restoration of normal haematopoiesis.
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.
Richard
Shambavi
S
Division of Hematology, Department of Medicine, Mount Sinai Medical Center, New York, NY 10029, USA.
Isola
Luis
L
Scigliano
Eileen
E
Singh
Harvinder
H
Najfeld
Vesna
V
Gilbert
Harriet
H
Weinberg
Rona S
RS
Fruchtman
Steven
S
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