Polycythaemia vera-transformation to myelofibrosis and subsequent reversal

A woman aged 67 who developed polycythaemia vera 21 years ago has been seen regularly since the diagnosis was first made. She was treated initially with pyrimethamine and then for 10 years by repeated venesections. After a 4 year period when her myeloproliferative disease was considered to be transitional a complete transformation to myelofibrosis occurred. She was treated by splenic irradiation and later with alkylating agents in an attempt to give her symptomatic relief from massive splenomegaly. Repeated and massive haemorrhage from gastro-oesophageal varices was treated initially by percutaneous transhepatic sclerosis of the gastro-oesophageal collateral vessels. A subsequent dacron mesocaval jump graft operation was successful in preventing further haematemesis and melaena. During the last year transformation back to polycythaemia vera has occurred. The evolution of her myeloproliferative disease has been fully documented by detailed blood counting, bone marrow aspirates and trephine biopsies, blood volume studies and other radioisotope investigations including the quantitation of functional erythropoietic tissue with 52Fe.