Rapidly developing myomatous erythrocytosis syndrome: a case report

Abstract

Myomatous erythrocytosis syndrome is polycythaemia associated with uterine leiomyoma, a rare condition known for over five decades with unclear aetiology. The present case is a 51-year-old Caucasian woman who presented with urinary retention and anaemia secondary to multiple uterine fibroids and menorrhagia 5 years following uterine artery embolisation. She opted for abdominal hysterectomy but preoperatively was found to be polycythaemic with haemoglobin of 23 g% and raised serum erythropoietin requiring serial venesections. At a year postoperatively she maintains normal haemoglobin and serum erythropoietin levels. Ectopic production of erythropoietin by fibroids is the most favoured aetiopathogenesis in this case. However, the rapid transition from anaemia to polycythaemia in 5 months is a striking feature as fibroid-linked polycythaemia has so far been reported on presentation. Vigilance is crucial in excluding polycythaemia while such patients are on waiting list as there is a risk of thromboembolism, which is further increased by surgery.