Myomatous erythrocytosis syndrome is polycythaemia associated with uterine fibroids. Although it is described in the literature for over five decades, the aetiology is still not clear. Ectopic production of erythropoietin by the leiomyoma is the most favoured aetiopathogenesis. Several studies using immunostaining techniques have confirmed the production of erythropoietin by the myoma tissue.2,3
A recent case report was published providing further evidence for in vivo erythropoietin production by the myomatous uterus where the erythropoietin levels in the uterine vein and artery4
were measured showing large gradient. Other proposed mechanisms for polycythaemia include renal production of erythropoietin and tissue hypoxia causing arteriovenous shunting in the myoma tissue.5
Gonadotrophin-releasing hormone (GnRH) analogues are commonly used preoperatively to control anaemia secondary to menorrhagia or shrink uterine fibroids to facilitate surgery. There is no published association between the use of GnRH analogues and hypererythropoietinaemia.
In our case, the most likely cause of raised erythropoietin level is excessive production by the fibroids as the levels were increased despite adequate menorrhagia control and correction of anaemia. Furthermore, the serum erythropoietin levels rapidly returned to normal postoperatively while the haemoglobin and haematocrit levels remained at normal levels 1 year postoperatively. It is, however, difficult to ascertain whether the excessive production of erythropoietin has occurred acutely (shortly before establishing the diagnosis) or it was a longstanding condition masked by menorrhagia (autovenesection).
Another interesting feature is that the rapid transition from anaemia to polycythaemia within a span of 5 months; unlike in all the case reports published so far where the polycythaemia was associated with fibroids to begin with on presentation.
Therefore, all clinicians need to be vigilant in excluding polycythaemia while such patients are on the waiting list and, more importantly, preoperatively as surgery will further increase the risk of thromboembolism.
- Myomatous erythrocytosis syndrome is polycythaemia associated with uterine fibroids.
- The existence of this condition should not be ignored and vigilance is crucial in excluding polycythaemia, especially in patients with giant leiomyomata.
- Polycythaemia is associated with high risk of thromboembolism, which is further increased by surgery, and optimisation of the haemoglobin levels is important prior to considering surgery.