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

Myomatous erythrocytosis syndrome is a rare phenomenon of secondary polycythemia evolving from uterine leiomyoma. Although the underlying pathology is still unknown, patients have an increased risk of venous thrombosis. A 44-year-old GO (gravida zero) presented with an incidental finding of secondary polycythemia, and a diagnosis of myomatous erythrocytosis syndrome was made because of her large uterine fibroids. She was placed on therapeutic anticoagulation after developing pulmonary embolisms and a dural sinus venous thrombosis. Subsequently, she underwent uterine artery embolization, which resulted in a substantial decrease in her erythropoietin (8.1 mU/mL) along with hemoglobin (15.1 g/dL) and hematocrit (4 5g/dL). Myomatous erythrocytosis syndrome can cause venous thrombosis, leading to neurologic complications. In patients with increased risk for surgery, uterine artery embolization is an effective option for treatment.

SURPRISING ERYTHROCYTOSIS RESOLUTION AFTER MYOMECTOMY: MYOMATOUS ERYTHROCYTOSIS SYNDROME.

Myomatous erythrocytosis syndrome (MES) is a rare gynaecological condition associated with the presence of a uterine fibroid and isolated polycythaemia. The main characteristic of MES is that haemoglobin returns to a normal level after removal of the myoma. MES is poorly known and still not fully understood. This case report describes the history of a patient with MES with surprising erythrocytosis resolution after myomectomy. LEARNING POINTS: A case of myomatous erythrocytosis syndrome with a normal erythropoietin level is described, adding to the scarce literature; the mediator responsible for the erythrocytosis is not yet known.This is a rare presentation of a common condition: erythrocytosis and uterine fibroids are frequent in the general population but are only anecdotally correlated.Delayed diagnosis led to unnecessary aggressive treatment with the aim of reducing the haematocrit level to below 45%.

Case Report: Myomatous erythrocytosis syndrome presenting as rapid growth of an extra-uterine mass.

Objective: To report a case of myomatous erythrocytosis syndrome (MES) with an extra-uterine manifestation. Case report: A 43-year-old woman presented with progressive abdominal distension and rapid enlargement of a pelvic mass. Upon survey, a high-level of hemoglobin (19.0 g/dl) was documented. The initial impression was an ovarian malignancy, but uterine sarcoma could not be ruled out because of its rapid growth. However, during exploratory laparotomy, the pelvic mass was found to be a 31 cm broad ligament leiomyoma; which is extremely rare for its size and location. The specimen was further studied immunohistochemically, which revealed excessive expressions of erythropoietin and erythropoietin receptors in addition to the diffusely matured blood vessels in the myoma tissue. The patient's hemoglobin level resumed to normal three months post-surgery. The diagnosis of MES was confirmed both clinically and histologically. Conclusion: A correct preoperative diagnosis is challenging when MES manifests as an extrauterine mass. The coexistence of MES should be considered in the management of all leiomyoma with polycythemia, regardless of locations.

Myomatous erythrocytosis syndrome: A case report.

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 × 1012/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.

Myomatous Erythrocytosis Syndrome: Case Report and Review of the Literature.

Myomatous Erythrocytosis Syndrome is defined as erythrocytosis, myomatous uterus, and the return of normal hematologic values following surgical resection. The exact role of erythropoietin in disease pathogenesis is unknown. In this study we report the case of a 49 year old premenopausal woman who was found to have an enlarged heterogeneous mass arising from the uterus concerning for malignancy. Her RBC count was 5.75 T/L, hemoglobin was 17.6 g/dL and hematocrit was 54.3%. Pre-operative erythropoietin levels were 24.6 mIU/mL and JAK2 mutation was not detected. She underwent Total Abdominal Hysterectomy and Bilateral Salpingo-Oophorectomy. The pathology was consistent with a uterine leiomyoma. Laboratory evaluation performed eight weeks after surgery showed a RBC count of 4.5 T/L, hemoglobin of 13.6 g/dL, hematocrit of 40.5%. Post-operative erythropoietin level was 5.4 mIU/mL. The tissue showed diffuse moderate to strong cytoplasmic immunopositive for Erythropoietin. Erythropoietin plays an important role in this condition, however the exact mechanism is still under investigation. The theory of erythropoietin secreting tumor autonomously without negative feedback is the most credible so far. However, further studies with use of blood erythropoietin level, tissue erythropoietin detection using immune-stain and new molecular biology techniques need to be done and compared to uterine myoma patients with no erythrocytosis. Usually, no further treatment is required following surgical removal.

Laparoscopic hysterectomy as optimal approach for 5400 grams uterus with associated polycythemia: A case report.

BACKGROUND: Uterine myomas are the most common benign gynecological soft tissue tumors in women but polycythemia associated with these tumors is rare. Polycythemia is significantly associated with risk of venous thromboembolism (VTE), which is further increased in case of a large pelvic mass and obesity. Here we report the surgical challenges in the case of an obese patient with a giant fibromatous uterus and associated polycythemia. CASE SUMMARY: A 42-year-old obese woman was referred to our department for treatment for a huge fibromatous uterus. She suffered of pelvic pressure and abdominal distension and reported severe dyspnea from several months. Laboratory analyses including hemoglobin (Hb) 19.2 g/dL and hematocrit (Hct) 59.7% were indicative of polycythemia. Arterial blood gas analysis showed arterial oxygen pressure (pO2) of 81.5 mmHg. Abdominal computed tomography confirmed a giant fibromatous uterus measuring 28.2 cm × 17 cm × 25 cm. To reduce the thromboembolic risk, the patient underwent low molecular weight heparin, phlebotomy twice before surgery, and we opted for a laparoscopic hysterectomy. The uterus weighed 5400 g and the histology confirmed a diagnosis of leiomyoma. We did not find increased erythropoietin levels in the supernatant of the myomatous uterine tissue. There were no complications. On postoperative day 1, the Hb and the Hct levels normalized to 13.3 g/dL and 41.7%, respectively. Arterial blood gas analysis after surgery showed a pO2 of 144.7 mmHg. These results suggested the diagnosis of myomatous erythrocytosis syndrome. The patient was discharged on the second postoperative day in very good condition with no symptoms. CONCLUSION: We believe this is the first reported laparoscopic hysterectomy in a patient with a giant uterus, polycythemia and obesity. Multiple VTE risk factors warranted a laparoscopic approach.

Aberrant expression of erythropoietin in uterine leiomyoma: implications in tumor growth.

OBJECTIVE: Myomatous erythrocytosis syndrome is a rare complication of uterine leiomyoma caused by erythropoietin (EPO) that is produced by tumor cells. We assessed the EPO expression in leiomyomas and investigated the effects of EPO on the tumor growth. STUDY DESIGN: Tissue samples were collected from 114 patients with uterine leiomyomas who underwent myomectomy or hysterectomy in Yokohama City University Hospital. From 17 patients, the corresponding normal myometrium was also collected. All samples were analyzed for EPO messenger RNA (mRNA) expression by real-time reverse transcription-polymerase chain reaction. EPO protein expression was determined by an enzyme-linked immunosorbent assay. The relationships between EPO expression and clinicopathological features were retrospectively analyzed using the patients' charts. Blood vessel density and maturity were assessed using hematoxylin-eosin staining and CD34 immunohistochemistry. RESULTS: EPO mRNA expression was detected in 108 of 114, or 95%, of the leiomyomas. The mean EPO mRNA expression in the leiomyoma was higher than the corresponding normal myometrium (3836 ± 4122 vs 1455 ± 2141; P = .025 by Wilcoxon rank test). The EPO mRNA expression in the leiomyomas varied extensively among samples, ranging from undetectable levels to 18-fold above the mean EPO mRNA of normal myometrium. EPO protein production was observed concomitant with mRNA expression. A positive correlation of leiomyoma size and EPO mRNA expression was shown by Spearman rank correlation coefficient (ρ = 0.294; P = .001), suggesting the involvement of EPO in leiomyoma growth. The blood vessel maturity was also significantly increased in EPO-producing leiomyomas (high vessel maturity in high vs low EPO group: 67% vs 20%; P = .013 by Fisher exact test). CONCLUSION: This report demonstrates that EPO is produced in most of conventional leiomyomas and supports a model in which EPO accelerates tumor growth, possibly by inducing vessel maturity. Our study suggests one possible mechanism by which some uterine leiomyomas reach a large size, and the understanding of EPO expression patterns in these tumors may be useful for management of the patients with leiomyomas.