Transition of myelofibrosis to polycythaemia vera

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.