The Myomatous Erythrocytosis Syndrome

Abstract

Uterine leiomyomas are the most common benign pelvic tumours in females.Rarely they are associated with symptoms of ectopic hormone production. One of thesesyndromes is the myomatous erythrocytosis syndrome, which is a triad of polycythaemia,fibroid(s) and resolution of the polycythaemia after hysterectomy. The case presented is of apost-menopausal lady found to have a Hb of 21 during workup of a pelvic mass. A diagnosis ofsecondary polycythemia due to ectopic erythropoietin production by the fibroid was made. Afterfive sessions of phlebotomiesa target Hb of 15 was obtained for surgery. The haematologicalvalues restored to normal three days post-operatively. It has been postulated that all fibroidsproduce erythropoietin. Recognition and proper management of this condition is importantto differentiate between primary and secondary polycythaemia as primary polycythemiais a thromboembolic condition, to avoid un-necessary investigations and to prevent anycomplications secondary polycythaemia may have per-operatively.