A Case of Myomatous Erythrocytosis Syndrome Associated with a Large Uterine Leiomyoma

Several etiologies have been proposed for erythrocytosis associated with uterine leiomyoma. We report a case of erythrocytosis associated with a large uterine leiomyoma, in which specific immunostaining for erythropoietin was positive. A 55-year-old woman, gravida 0, para 0, was referred to our hospital for treatment for a large uterine myoma and erythrocytosis. She had no vaginal bleeding after she reached menopause at 50 years old. She had severe polycythemia: hemoglobin (Hb), 19.9？g/dL; red blood cell count (RBC), 6.65 × 106/mm3; hematocrit, (Hct) 59.1%. An abdominal simple hysterectomy was performed, and a pathological examination confirmed the diagnosis of leiomyoma of the uterus. In addition, immunostaining demonstrated that the cytoplasm of the leiomyoma cells was strongly positive for erythropoietin. After the operation, the patient’s hemoglobin and hematocrit levels normalized, and we diagnosed her condition as myomatous erythrocytosis syndrome. 1. Introduction Ectopic erythropoietin production is known to occur as a complication of various tumors such as renal cell carcinoma, hepatocellular carcinoma, and cerebellar hemangioblastoma. However, it is less well known that it can also occur as a complication of uterine leiomyoma. Since Thomson and Marson first described myomatous erythrocytosis syndrome in 1953 [1], the etiology of the syndrome has been investigated using a range of methods, and various etiologies have been proposed [2, 3]. 2. Case A 55-year-old woman, gravida 0, para 0, was referred to our department for treatment for a large uterine myoma. She presented with hyperhemoglobinemia, and hematological examinations produced the following results: hemoglobin, 19.9？g/dL; hematocrit, 59.1%; white blood cell count, 7,900/mm3; red blood cell count, 6.65 × 106/mm3; and serum levels of erythropoietin, 28.5？mU/mL (normal range: 0–29.0？mU/mL). In pelvic examination, uterus size was newborn child head, the mobility of uterus was good, and she had no abdominal tenderness. The size of uterus was over 20？cm. And there was a giant intramural myoma which was cm × cm, in preoperative pelvic ultrasound examination. The result of preoperative cytology of the endometrium was normal though the preoperative diagnostic endometrial curettage was not performed. Her cardiovascular and respiratory systems were normal. The results of other examinations, including magnetic resonance imaging (MRI) of the head to find the cerebellar hemangioma which could produces erythropoietin, upper abdominal ultrasonography, and chest X-rays were all normal. We suspected