Myomatous erythrocytosis syndrome: Case report and literature review

Objective: To present a clinical case of Myomatous Erythrocytosis Syndrome (MES) and to review the literature regarding its diagnosis and treatment. Materials and methods: We describe the case of a 45-year-old woman who presented to a medium- complexity hospital with abnormal uterine bleeding. She was diagnosed with myomatosis and polycythemia. She underwent hysterectomy with resolution of erythrocytosis 42 days after surgery. A literature review was conducted through searches in the Medline, EMBASE, and LILACS databases using related MeSH and DeCS terms. Case reports and case series of women with MES were included, and information regarding diagnosis and treatment was collected from publications in English and Spanish from the last 20 years. Results: Twenty-nine titles were identified, of which 17 met the inclusion criteria, corresponding to 15 case reports and two case series that together described 20 patients with MES. The most frequent symptoms were progressive enlargement of the abdominal mass (85 %), pelvic pain (65 %), and abnormal uterine bleeding (35 %). Ultrasound was the most commonly used imaging technique, while molecular biology and hormone studies were infrequently performed. Hysterectomy was the most common treatment, with complete resolution of erythrocytosis in all cases with available follow-up. Few reports described complications associated with this condition. Conclusions: MES should be considered in the differential diagnosis of women with unexplained polycythemia and uterine leiomyomatosis. Hysterectomy constitutes the definitive treatment, leading to normalization of hematologic parameters. Further studies are needed to better understand its pathophysiology asses new therapeutic strategies and its complications.