Keywords
Leiomioma uterino
Eritropoietina
Policitemia secundária
How to Cite
Abstract
Background: Myomatous erythrocytosis syndrome (MES) is a rare condition characterized by uterine leiomyomas with secondary polycythemia. Despite isolated reports, its pathogenesis, clinical presentation and optimal management remain poorly defined. Methods: We performed a narrative review of 21 PubMed-indexed studies (1995–2025) on MES, extracting data on patient demographics, clinical manifestations, hematological parameters, immunohistochemical findings and therapeutic outcomes. Results: MES predominantly affects women around 49 years old with large uterine fibroids (>10 cm). Common presentations include abdominal distension, pelvic pain and hyperviscosity symptoms. Preoperative hemoglobin ranges from 17.6–23 g/dL; erythropoietin (EPO) levels are often elevated but may be normal in some cases. Immunohistochemical analyses demonstrate ectopic EPO and EPO-receptor expression within leiomyoma tissue, supporting an autocrine/paracrine erythropoiesis mechanism. Surgical resection (myomectomy or hysterectomy) and uterine artery embolization consistently normalize hemoglobin, hematocrit and EPO levels. Temporary phlebotomy is used to mitigate thrombotic risk prior to definitive treatment. Conclusions: MES should be considered in the differential diagnosis of unexplained secondary polycythemia in women with large uterine masses. Early recognition of the triad “large fibroid + isolated erythrocytosis + post-resection normalization” can prevent unnecessary interventions and reduce thromboembolic complications. Future multicenter trials and molecular studies are warranted to refine diagnostic biomarkers and compare therapeutic modalities.
References
ANSARI, F.; AL ASSIL, T.; OMAIRA, M.; HOEKSTRA, A. V. Myomatous erythrocytosis syndrome: a uterine fibroid associated with polycythaemia. BMJ Case Reports, v. 17, n. 3, e256927, 2024. DOI: https://doi.org/10.1136/bcr-2023-256927.
ASANO, R.; ASAI-SATO, M.; MIYAGI, Y. et al. Aberrant expression of erythropoietin in uterine leiomyoma: implications in tumor growth. American Journal of Obstetrics and Gynecology, v.213, n.2, p.199.e1-199.e8, 2015. DOI: 10.1016/j.ajog.2015.02.016.
BLOCKEEL, C.; DE BEECK, B. O.; BOURGAIN, C.; AMY, J. J. Myomatous erythrocytosis syndrome. National Medical Journal of India, v. 18, n. 5, p. 247–249, 2005. DOI: https://doi.org/10.4103/0970-258X.16433138.
COUILLANDRE, P.; LEWALLE, P.; BENGHIAT, S. F.; SALAROLI, A. Surprising erythrocytosis resolution after myomectomy: myomatous erythrocytosis syndrome. European Journal of Case Reports in Internal Medicine, v. 10, n. 3, 003722, 2023. DOI: https://doi.org/10.12890/2023_003722.
DE GUZMAN, G. S.; MANALO, E. M. Myomatous erythrocytosis syndrome: a case series. Case Reports in Women’s Health, v. 24, e00139, 2019. DOI: https://doi.org/10.1016/j.crwh.2019.e00139.
DE OLIVEIRA MARQUES, M.; GOMES, N. C.; DE ANDRADE YOUNG, P.; DE OLIVEIRA MARQUES, O.; KERBAUY, F. R.; SANDES, A. F. Myomatous erythrocytosis syndrome—an uncommon cause of secondary polycythemia. Annals of Hematology, v. 102, n. 8, p. 2275–2276, 2023. DOI: https://doi.org/10.1007/s00277-023-05176-8.
FEKETE, S.; SOMORÁCZ, Á.; RADNAI, K.; VILIMI, B.; BABARCZI, E. [The pathogenic role of uterine erythropoietin in a case of myomatous erythrocytosis syndrome]. Orvosi Hetilap, v. 163, n. 52, p. 2088–2092, 2022. DOI: https://doi.org/10.1556/650.2022.32641.
GORDON, B.; FISCHBECK, T.; SALAMO, R.; SCHROFF, S. Treating myomatous erythrocytosis syndrome with uterine artery embolization. Obstetrics & Gynecology, v. 133, n. 6, p. 1274–1277, 2019. DOI: https://doi.org/10.1097/AOG.0000000000003289.
KOHAMA, T.; SHINOHARA, K.; TAKAHURA, M.; INOUE, M. Large uterine myoma with erythropoietin messenger RNA and erythrocytosis. Obstetrics and Gynecology, v.96, n.5 Pt 2, p.826-828, 2000. DOI: 10.1016/S0029-7844(00)00952-2.
LEVGUR, M.; LEVIE, M. D. The myomatous erythrocytosis syndrome: a review. Obstetrics and Gynecology, v. 86, n. 6, p. 1026–1030, 1995. DOI: https://doi.org/10.1016/0029-7844(95)00292-Y.
MUI, J.; YANG, M. M. H.; COHEN, T.; McDONALD, D. I.; HUNT, H. More than a myoma: a review of myomatous erythrocytosis syndrome. Journal of Obstetrics and Gynaecology Canada, v. 42, n. 2, p. 198–203.e3, 2020. DOI: https://doi.org/10.1016/j.jogc.2018.12.025.
NARITA, F.; OHARA, N.; FUKUNAGA, K. Myomatous erythrocytosis syndrome. Journal of Obstetrics and Gynaecology, v. 23, n. 5, p. 577, 2003. DOI: https://doi.org/10.1080/0144361031000156654.
NHALIL, K. V.; D’COUTH, S.; MAROLI, R.; AMBILI, K.; RAHMAN, K. P. S. Myomatous erythrocytosis syndrome in pregnancy managed with classical caesarean section and myomectomy: a case report. Journal of Obstetrics and Gynaecology India, v. 71, n. 3, p. 322–325, 2021. DOI: https://doi.org/10.1007/s13224-021-01447-4.
ONO, Y.; HIDAKA, T.; FUKUTA, K.; KOUCHI, K.; YASOSHIMA, K.; TAKAGAWA, K.; ARAI, T. A case of myomatous erythrocytosis syndrome associated with a large uterine leiomyoma. Case Reports in Obstetrics and Gynecology, 2014:602139, 2014. DOI: https://doi.org/10.1155/2014/602139.
OZSARAN, A. A.; ITIL, I. M.; TEREK, C.; KAZANDI, M.; DIKMEN, Y. Giant myoma and erythrocytosis syndrome. The Australian & New Zealand Journal of Obstetrics and Gynaecology, v.39, n.3, p.384-386, 1999. DOI: 10.1111/j.1479-828X.1999.tb03426.x.
PADAVALA, J.; ABDELMAGIED, A.; EMERY, S. Rapidly developing myomatous erythrocytosis syndrome: a case report. BMJ Case Reports, 2010;bcr11.2009.2443, 2010. DOI: https://doi.org/10.1136/bcr.11.2009.2443.
PATEL, P. B.; RADIN, R. Myomatous erythrocytosis syndrome: a case report. Acta Radiologica, v. 47, n. 9, p. 998–999, 2006. DOI: https://doi.org/10.1080/02841850600851742.
POLLIO, F.; STAIBANO, S.; MANSUETO, G.; DE ROSA, G.; PERSICO, F.; DE FALCO, M.; DI LIETO, A. Erythropoietin and erythropoietin receptor system in a large uterine myoma of a patient with myomatous erythrocytosis syndrome: possible relationship with the pathogenesis of unusual tumor size. Human Pathology, v. 36, n. 1, p. 120–127, 2005. DOI: https://doi.org/10.1016/j.humpath.2004.10.009.
SHU, X. Y.; CHEN, N.; CHEN, B. Y.; YANG, H. X.; BI, H. Myomatous erythrocytosis syndrome: a case report. World Journal of Clinical Cases, v. 10, n. 10, p. 3206–3212, 2022. DOI: https://doi.org/10.12998/wjcc.v10.i10.3206.
SURESH, P.; RIZK, S. Myomatous erythrocytosis syndrome: case report and review of the literature. Cureus, v. 12, n. 2, e6892, 2020. DOI: https://doi.org/10.7759/cureus.6892.
UNOSAWA, S.; HATA, M.; SEZAI, A.; NIINO, T.; YOSHITAKE, I.; MINAMI, K. Pulmonary embolism with myomatous erythrocytosis syndrome and extreme obesity. The Thoracic and Cardiovascular Surgeon, v. 57, n. 5, p. 313–314, 2009. DOI: https://doi.org/10.1055/s-2008-1038880.
VALENTE, E.; ZUEGER, M.; DONATO, D. A rare case of venous sinus thrombosis and pulmonary embolisms secondary to myomatous erythrocytosis syndrome. AJOG Global Reports, v. 4, n. 2, 100340, 2024. DOI: https://doi.org/10.1016/j.xagr.2024.100340.
VLASVELD, L. T.; DE WIT, C. W.; VERWEIJ, R. A.; CASTEL, A.; JANSEN, P. M.; PETERS, A. A. Myomatous erythrocytosis syndrome: further proof for the pathogenic role of erythropoietin. Netherlands Journal of Medicine, v. 66, n. 7, p. 283–285, 2008. PMID: 18663256.
WANG, S. J.; CHEN, Y. A.; SHIH, Y. H.; CHEN, M. J.; LU, C. H. Case report: myomatous erythrocytosis syndrome presenting as rapid growth of an extra-uterine mass. Frontiers in Surgery, v. 9, 950358, 2022. DOI: https://doi.org/10.3389/fsurg.2022.950358.
YOKOYAMA, Y.; SHINOHARA, A.; HIROKAWA, M.; MAEDA, N. Erythrocytosis due to an erythropoietin-producing large uterine leiomyoma. Gynecologic and Obstetric Investigation, v.56, n.4, p.179-183, 2003. DOI: 10.1159/000074104.
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2025 Journal of Advanced Clinical Implementation and Applied Medical Innovations (ISSN 3085-8348)