Abstract
BACKGROUND Uterine fibroids are common benign gynecologic tumors characterized by abnormal uterine bleeding, menorrhagia, and anemia. Myomatous erythrocytosis syndrome (MES), a rare secondary erythrocytosis associated with fibroids, presumably results from ectopic erythropoietin production by the fibroids, which activates the Janus kinase 2 (JAK-2) pathway and increases red blood cell counts. MES clinically presents with abdominal distension, skin discoloration, and menstrual irregularities. Management is complex due to substantial risks of intraoperative bleeding and thromboembolism. This report describes a 33-year-old woman with MES. After myomectomy, her erythrocytosis normalized, but she developed postoperative bleeding. CASE REPORT A 33-year-old woman presented with a 2-year history of progressive abdominal distension and an abdominal mass comparable in size to a 20-week gravid uterus, plethoric facies, and reddish-purple nail beds. Her hemoglobin level was 19.7 g/dL; hematocrit was 60.4%. Ultrasound and computed tomography confirmed a 25×20×12 cm fibromatous uterus. To reduce thromboembolic risk, she received low-molecular-weight heparin and intravenous hydration preoperatively. Abdominal myomectomy was performed, during which substantially increased uterine and fibroid vascularity was observed. However, the patient experienced severe postoperative hemorrhage (1800 mL/24 h), warranting a second operation for hemostasis. She recovered well and was discharged on postoperative day 14; her hemoglobin level normalized. Pathologic examination confirmed uterine leiomyoma, supporting the diagnosis of MES. CONCLUSIONS MES is a rare condition characterized by large uterine fibroids and erythrocytosis; postoperative bleeding and thrombosis are common complications. This case highlights the importance of accurate diagnosis and effective management. Erythropoietin-related therapeutic targets for MES are needed.