Abstract
BACKGROUND: Myomatous erythrocytosis syndrome is an uncommon paraneoplastic syndrome of erythrocytosis associated with benign uterine leiomyomas. Fewer than 70 cases have been reported globally, and clinical awareness remains limited. CASE PRESENTATION: We report the case of a 45-year-old Middle Eastern premenopausal woman who presented with a several-month history of abdominal distension and a hemoglobin level of 22.5 g/dL. Imaging revealed a massive subserosal uterine leiomyoma (190 × 181 × 115 mm). Following total abdominal hysterectomy with left salpingo-oophorectomy, her hemoglobin level normalized to 15 g/dL within days. Histopathological analysis revealed no evidence of malignancy, consistent with a diagnosis of myomatous erythrocytosis syndrome. CONCLUSION: Myomatous erythrocytosis syndrome should be considered in women presenting with unexplained erythrocytosis and large uterine leiomyomas. Timely diagnosis and surgical intervention can result in hematologic normalization and help prevent life-threatening thromboembolic complications.