Spinal cord compression secondary to extramedullary haematopoiesis in transformed polycythaemia rubra vera

Abstract

Polycythaemia vera (PV) is a clonal proliferative disorder of the bone marrow characterised by autonomous haematopoiesis, which results in a panmyelosis in the peripheral blood. It is typically characterised by an acquired mutation in JAK-2 V617F. Progression to myelofibrosis (MF), characterised by worsening cytopenias and the development of constitutional symptoms, is seen in up to 10% of cases. Extramedullary haematopoiesis (EMH) in the spleen is a common finding in myelofibrotic transformation, but elsewhere in the body it is extremely unusual. We report the case of a 69-year-old male whose PV progressed to secondary MF and who presented with compression of the thoracic spinal cord directly as a result of EMH. Cytogenetic and molecular findings in the bone marrow were in keeping with evolving myeloid disease. He was managed by surgical laminectomy with an excellent outcome. Extramedullary haematopoiesis may be seen in both PV and on transformation to MF. This very rare complication should be borne in mind when managing patients with myeloproliferative disorders.