THE MYOMATOUS ERYTHROCYTOSIS SYNDROME

A CASE REPORT AND REVIEW OF LITERATURE

Abstract

Uterine leiomyomas are the most common benign pelvic tumours in females.
Rarely they are associated with symptoms of ectopic hormone production. One of these
syndromes 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 a
post-menopausal lady found to have a Hb of 21 during workup of a pelvic mass. A diagnosis of
secondary polycythemia due to ectopic erythropoietin production by the fibroid was made. After
five sessions of phlebotomiesa target Hb of 15 was obtained for surgery. The haematological
values restored to normal three days post-operatively. It has been postulated that all fibroids
produce erythropoietin. Recognition and proper management of this condition is important
to differentiate between primary and secondary polycythaemia as primary polycythemia
is a thromboembolic condition, to avoid un-necessary investigations and to prevent any
complications secondary polycythaemia may have per-operatively.