Polycythaemia rubra vera and myelofibrosis with spinal cord compression.

Spinal cord compression from extramedullary haemopoiesis within the spinal epidural space is a rare complication of myelofibrosis and polycythaemia rubra vera (PRV). A 69-year-old male with PRV (later transforming to myelofibrosis) who developed this complication is described here. Due to the uncertainty over its optimal treatment, previous case reports were systematically reviewed to define its presentations, treatments and outcomes. Including the present case this complication has been reported in 21 patients with myelofibrosis and PRV: 17 were male and the mean symptom duration was 7.6 months. Neurological improvement occurred in 14 patients and 12 survived. Seventy-five per cent of patients receiving combined treatment (irradiation with laminectomy or chemotherapy) showed neurological improvement and 100% survived. In contrast, 67% of those receiving single treatments exhibited improved neurology and only 33% survived. It is concluded that spinal cord compression in myelofibrosis and PRV has a high mortality, with combined treatment providing a better prognosis.