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Syngeneic stem cell transplant for spent‐phase polycythaemia vera: eradication of myelofibrosis and restoration of normal haematopoiesis
Author(s) -
Richard Shambavi,
Isola Luis,
Scigliano Eileen,
Singh Harvinder,
Najfeld Vesna,
Gilbert Harriet,
Weinberg Rona S.,
Fruchtman Steven
Publication year - 2002
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.2002.03387.x
Subject(s) - polycythaemia , myelofibrosis , medicine , haematopoiesis , stem cell , bone marrow , splenectomy , transplantation , polycythemia vera , immunology , pathology , spleen , biology , genetics
Summary. 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.