Myomatous erythrocytosis syndrome: A case report

Abstract

BACKGROUND Uterine myoma is the most common benign tumor among women and is often accompanied by anemia. Here, we report the case of a patient with a very large leiomyoma but with a hemoglobin level as high as 197 g/L. After undergoing hysterectomy, all her hematological parameters returned to normal. Immunohistochemical staining of her myoma for erythropoietin showed strong positivity, which suggested that erythropoietin may be the cause of her erythrocytosis. A multidisciplinary team played a significant role in treating the disease. CASE SUMMARY A 47-year-old woman visited our department complaining that her abdomen had been continuously growing for the past 2 years. After careful examinations, she was suspected of having a very large leiomyoma. She was also diagnosed with erythrocytosis because her RBC count was 6.49 × 10 12 /L, hemoglobin was 197 g/L. Following a multidisciplinary team consultation, bilateral ureteral stents were placed, and 800 mL blood was removed by phlebotomy. The patient then underwent hysterectomy and bilateral salpingectomy. She recovered well from the operation, and her hemoglobin level decreased sharply following the surgery. Low-molecular-weight heparin was administered daily to prevent postoperative thrombosis. She was discharged from the hospital on the fourth postoperative day. Two months later, all her hematological parameters returned to normal. Pathological analysis of the myoma revealed that it was a benign leiomyoma, with partial hyalinization, and strong positivity for erythropoietin in immunohistochemical staining suggested that erythropoietin may be responsible for the erythrocytosis. CONCLUSION Erythropoietin ectopically produced from the myoma was responsible for the erythrocytosis in this patient. A multidisciplinary team is strongly recommended.

A rare case of venous sinus thrombosis and pulmonary embolisms secondary to myomatous erythrocytosis syndrome

“…Traditionally, MES is diagnosed by immunostaining for EPO. 16 Although pathologic sampling and staining were not conducted in this case, the researchers believe that a diagnosis of MES is warranted because the patient's laboratory values significantly dropped following UAE and remained at baseline following the removal of her uterus. It is important to note that this patient was on an oral contraceptive pill, which can increase the risk of VTE and stroke; therefore, OCP (Oral Contraceptive Pill) use must be considered as a possible cofounder.…”

Secondary Erythrocytosis Associated with Uterine Myoma Is Rare but Should Be of Concern

“…Evidence supports an increase in EPO levels in MES cases [5,9]. However, from the literature review of Mui et al [5], the presence of hydronephrosis was not different among MES cases, as well as clinical dyspnea or hypoxia was not always seen [2,10]. In addition, this makes this theory less reliable.…”

“…Recently, Suresh and Rizk [9] discovered positivity of EPO immunohistochemistry in fibroid tissue, and the authors detected an increase in EPO level in blood serum, which returned to normal levels after removal of the mass. Moreover, Shu et al [10] found that EPO immunohistochemical staining was strongly positive in the myoma tissue, which may be the cause of erythrocytosis. This led to the hypothesis that EPO production from fibroids is responsible for MES.…”