Pleural effusion secondary to extramedullary haemopoiesis in a patient with idiopathic myelofibrosis responding to pleurodesis and hydroxyurea.

Abstract

A case of idiopathic myelofibrosis presenting with a pleural effusion secondary to extramedullary haemopoeisis is described. Approximately 2 years following the diagnosis of his myeloprolioferative disorder the patient presented with dyspnoea. Physical signs were consistent with a pleural effusion which was confirmed radiologically. Cytology of the effusion fluid demonstrated myeloid precursors, including megakaryocytes. The effusion required repeated draining and a pleurodesis was undertaken in an attempt to prevent reaccumulation of the fluid. The procedure was successful and follow up over a period of 5 months demonstrated no recurrence of the pleural effusion on that side of the chest. An effusion later occurred on the other side but was managed conservatively by drainage. Hydroxyurea was introduced at that stage, but shortly afterwards the patient died from an unrelated cause. We review the literature on this uncommon complication of myelofibrosis and discuss the options available to treat the disorder.