More Than a Myoma: A Review of Myomatous Erythrocytosis Syndrome

Mui, Justin; Yang, Michael; Cohen, Trevor; McDonald, David I.; Hunt, Harold E.

doi:10.1016/j.jogc.2018.12.025

Cited by 13 publications

(35 citation statements)

References 28 publications

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“…An echocardiogram with shunt study is indicated in the presence of relevant cardiac findings. In females, myomatous erythrocytosis syndrome should be kept in mind, particularly since erythrocytosis is often masked by menorrhagia [ 86 ]. Rare acquired disorders to consider include the monoclonal gammopathy driven TEMPI syndrome which is characterized by telangiectasias, elevated serum EPO (which might be >5000 mIU/ml) and erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting [ 87 , 88 ]; erythrocytosis and telangiectasis are often the initial manifestation, therefore obtaining monoclonal protein studies might provide diagnostic clues to this entity.…”

Section: Diagnostic Approachmentioning

confidence: 99%

JAK2 unmutated erythrocytosis: current diagnostic approach and therapeutic views

et al. 2021

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JAK2 unmutated or non-polycythemia vera (PV) erythrocytosis encompasses both hereditary and acquired conditions. A systematic diagnostic approach begins with documentation of historical hematocrit (Hct)/hemoglobin (Hgb) measurements and classification of the process as life-long/unknown duration or acquired. Further investigation in both categories is facilitated by determination of serum erythropoietin level (EPO). Workup for hereditary/congenital erythrocytosis requires documentation of family history and laboratory screening for high-oxygen affinity hemoglobin variants, 2, 3 biphosphoglycerate deficiency, and germline mutations that are known to alter cellular oxygen sensing (e.g., PHD2, HIF2A, VHL) or EPO signaling (e.g., EPOR mutations); the latter is uniquely associated with subnormal EPO. Acquired erythrocytosis is often elicited by central or peripheral hypoxia resulting from cardiopulmonary disease/high-altitude dwelling or renal artery stenosis, respectively; EPO in the former instance is often normal (compensated by negative feed-back). Other conditions associated with acquired erythrocytosis include EPO-producing tumors and the use of drugs that promote erythropoiesis (e.g., testosterone, erythropoiesis stimulating agents). “Idiopathic erythrocytosis” loosely refers to an otherwise not explained situation. Historically, management of non-PV erythrocytosis has been conflicted by unfounded concerns regarding thrombosis risk, stemming from limited phenotypic characterization, save for Chuvash polycythemia, well-known for its thrombotic tendency. In general, cytoreductive therapy should be avoided and phlebotomy is seldom warranted where frequency is determined by symptom control rather than Hct threshold. Although not supported by hard evidence, cardiovascular risk optimization and low-dose aspirin use are often advised. Application of modern genetic tests and development of controlled therapeutic intervention trials are needed to advance current clinical practice.

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Section: Diagnostic Approachmentioning

confidence: 99%

JAK2 unmutated erythrocytosis: current diagnostic approach and therapeutic views

et al. 2021

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“…Under rare conditions, secondary erythrocytosis can originate from benign tumors, such as uterine myoma[ 4 ], which is called myomatous erythrocytosis syndrome. As myoma may cause abnormal uterine bleeding, which leads to fluctuations in hemodynamics such as anemia, the actual occurrence of MES may be higher than reported[ 5 , 6 ].…”

Section: Discussionmentioning

confidence: 92%

“…Recently, Mui et al [ 5 ] reported a case of MES and summarized all 55 previous similar cases. In his study, the mean age at presentation was 48.7 ± 12.3 years, and there was no difference in parity or menopausal status among the patients in the reported cases.…”

Section: Discussionmentioning

confidence: 99%

Myomatous erythrocytosis syndrome: A case report

Shu¹,

Chen²,

Yang³

et al. 2022

WJCC

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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.

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“…Los tres criterios se observaron en los casos conocidos hasta el día de hoy. [2][3][4] Hasta la fecha se han notificado menos de 40 casos en todo el mundo. Los leiomiomas o fibromas uterinos son los tumores sólidos más comunes en mujeres.…”

Section: Wwwmedigraphicorgmx Introducciónunclassified

Eritrocitosis y miomatosis

Troche¹

2020

Revista CONAMED

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Los leiomiomas uterinos son tumores asociados a tumores de eritrocitosis secundaria. En la literatura se ha propuesto y demostrado la producción de eritropoyetina ectópica por fibromas de músculos lisos. Se ha demostrado que la eritrocitosis aislada es el resultado de la producción ectópica de eritropoyetina (Epo) en varias neoplasias malignas que incluyen células renales y carcinoma hepatocelular. Se ha encontrado que ocurre en tumores extraños como el hemangioblastoma cerebeloso y los adenomas. Se le asocia con menos frecuencia a leiomioma uterino. Los cirujanos emplean cada vez más la laparoscopia para la histerectomía. Sin embargo, el papel de la laparoscopia en presencia de un útero gigante todavía es debatible. En pacientes con síndrome miomatoso eritrocitario (SME) presente con signos y síntomas atribuidos a una masa abdominopélvica o eritrocitosis la flebotomía preoperatoria ayuda a disminuir la gravedad de la eritrocitosis y se ha utilizado para mitigar el riesgo de complicaciones trombóticas. La resección quirúrgica del leiomioma es un enfoque válido para el tratamiento de SME.

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More Than a Myoma: A Review of Myomatous Erythrocytosis Syndrome

Cited by 13 publications

References 28 publications

JAK2 unmutated erythrocytosis: current diagnostic approach and therapeutic views

JAK2 unmutated erythrocytosis: current diagnostic approach and therapeutic views

Myomatous erythrocytosis syndrome: A case report

Eritrocitosis y miomatosis

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