Transition of Myelofibrosis to Polycythaemia Vera

Abstract

A case of typical myelofibrosis with a huge spleen is described in a 62‐year‐old man. During the subsequent 2‐year follow‐up, a clinical picture of polycythaemia vera with pancytosis and disappearance of the marrow fibrosis was observed. The pancytosis necessitated treatment with busulphan and frequent phlebotomies. The transformation was associated with prednisone treatment for a suspected haemolytic state. During this treatment, the spleen no longer became enlarged on clinical palpation, although it was still enlarged at post mortem examination, but much less than 2 years earlier.

Chemotherapy resolves symptoms and reverses marrow fibrosis in myelofibrosis

“…The possibility of this observation being due to sampling error was entertained, but was considered unlikely because of the length of the biopsy sections examined and that it would occur in each of the five patients. Resolution of bone marrow fibrosis in M F following chemotherapy has not been reported previously, although 2 recent papers have documented reversal from M F to polycythaemia Vera in patients with the so-called transitional myeloproliferative disorders (15,16). The pathogenesis of bone marrow fibrosis in M F is not clear, but recent studies suggest that it is a reaction by normal stromal cells to abnormal haemopoietic cells from a clonal proliferation, or their environment (17)(18)(19).…”

A sequential histological study of bone marrow fibrosis in idiopathic myelofibrosis

“…However, long-term cytotoxic therapy has been shown to be associated with rising Hb-concentration in concert with regression of bone marrow fibrosis and clinical improvement (14,17). Several of our patients were also treated with prednisolone which, in at least 2 cases was associated with regression of bone marrow fibrosis and improvement in peripheral blood parameters (20,26,36).…”

“…Fourth, spontaneous reversal of myelofibrosis (35) and transition of IMF to PV (13,36,37) may occur. Finally, regression of myelofibrosis may not only be mediated by cytotoxic (14,17,18,(21)(22)(23)(24)(25)27,(38)(39)(40) or immunosuppressive therapy (11,20,26,36,41), but has also been described following splenic irradiation (42), splenectomy (37,43) and in patients who enter a blastic transformation (13,14,44).…”

“…Material and methods 36 patients with IMF were studied. Most patients were included in clinical and collagen metabolic studies previously described (28)(29)(30)(31)(32)(33).…”

Polycythaemia Following Splenectomy in Myelofibrosis with Myeloid Metaplasia. A Reorganization of Erythropoiesis

“…In 1 case, polycythaemia appeared spontaneously early in the course of the disease (17), thus the case may be considered a variant of myeloid metaplasia of the polycythaemic type (18). In another case (19), an effect of prednisone on autoimmune mechanism of anaemia could not be excluded. Our 3 cases give additional evidence that the transformation of myeloid metaplasia into polycythaemia can be considered as a rare consequence of therapy, more than a spontaneous event in the course of the disease.…”