Medicine:Myomatous erythrocytosis syndrome

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Myomatous erythrocytosis syndrome
Other namesMES
SpecialtyHematology

Myomatous erythrocytosis syndrome (MES) is an uncommon gynecological disorder associated with isolated polycythemia and uterine fibroids. The primary feature of myomatous erythrocytosis syndrome is that hemoglobin goes back to its baseline level following the removal of the myoma. There have only been 50 cases of myomatous erythrocytosis syndrome documented as of 2023.[1] Thomsen and Marson published the first case in 1953.[2]

Signs and symptoms

The most typical presentations were dyspnea (12.3%), menstrual irregularities (24.6%), skin discoloration (33.3%), and abdominal or pelvic distension or mass (93%).[3]

Causes

Previous explanations for the etiology of myomatous erythrocytosis syndrome included the possibility of renal outflow obstruction,[4] myomatous arteriovenous shunts,[5] or interference with pulmonary ventilation[6] as possible pathways resulting in the hematological abnormalities that are characteristic of this illness. But these theories have been refuted.[7] The current theory regarding myomatous erythrocytosis syndrome links aberrant EPO production from the myomatous tissue to the observed hematological changes.[8][9]

Diagnosis

Hematological values such as hemoglobin, hematocrit, and red blood cell counts are elevated in patients with myomatous erythrocytosis syndrome.[3]

The diagnostic criteria for myomatous erythrocytosis syndrome includes polycythemia, uterine myoma, and return of hemoglobin levels to normal following fibroid excision surgery.[10]

Treatment

Hysterectomy is the most common treatment for myomatous erythrocytosis syndrome.[3] Horwitz and McKelway successfully performed the first myomectomy for myomatous erythrocytosis syndrome.[11] Perioperative bleeding and urogenital injuries were found to be the most frequent surgical complications in myomatous erythrocytosis syndrome cases, which are comparable to those encountered during the resection of a large myomatous uterus.[12]

See also

References

  1. ↑ Salaroli, Adriano; Couillandre, Pauline; Lewalle, Philippe; Benghiat, Samantha Fleur (March 18, 2023). "Surprising erythrocytosis resolution after myomectomy: myomatous erythrocytosis syndrome". European Journal of Case Reports in Internal Medicine (SMC Media) 10 (3): 003722. doi:10.12890/2023_003722. ISSN 2284-2594. PMID 36969520. 
  2. ↑ Thomson, A.P.; Marson, F.G.W. (1953). "POLYCYTHÆMIA WITH FIBROIDS". The Lancet (Elsevier BV) 262 (6789): 759–760. doi:10.1016/s0140-6736(53)91456-1. ISSN 0140-6736. 
  3. ↑ 3.0 3.1 3.2 Mui, Justin; Yang, Michael M.H.; Cohen, Trevor; McDonald, David I.; Hunt, Harold (2020). "More Than a Myoma: A Review of Myomatous Erythrocytosis Syndrome". Journal of Obstetrics and Gynaecology Canada (Elsevier BV) 42 (2): 198–203.e3. doi:10.1016/j.jogc.2018.12.025. ISSN 1701-2163. PMID 30904341. 
  4. ↑ Maudsley, R. F.; Boehm, F. H. (September 9, 1970). "Secondary polycythemia and uterine leiomyomata.". Canadian Medical Association Journal (Canadian Medical Association) 103 (5): 517–518. PMID 5469625. 
  5. ↑ Horwitz, Alec (August 13, 1955). "Polycythemia Associated with Uterine Myomas". Journal of the American Medical Association (American Medical Association (AMA)) 158 (15): 1360–1361. doi:10.1001/jama.1955.02960150030009. ISSN 0002-9955. PMID 13242355. 
  6. ↑ Paranjothy, D.; Vaish, S. K. (1967). "Polycythaemia Associated with Leiomyoma of the Uterus". BJOG: An International Journal of Obstetrics & Gynaecology 74 (4): 603–605. doi:10.1111/j.1471-0528.1967.tb04001.x. ISSN 1470-0328. PMID 6033281. 
  7. ↑ LEVGUR, M; DLEVIE, M (1995). "The myomatous erythrocytosis syndrome: A review". Obstetrics & Gynecology (Ovid Technologies (Wolters Kluwer Health)) 86 (6): 1026–1030. doi:10.1016/0029-7844(95)00292-y. ISSN 0029-7844. PMID 7501327. 
  8. ↑ Suzuki, Mitsuaki; Takamizawa, Satoru; Nomaguchi, Koji; Suzu, Shinya; Yamada, Muneo; Igarashi, Takashi; Sato, Ikuo (2001). "Erythropoietin synthesis by tumour tissues in a patient with uterine myoma and erythrocytosis". British Journal of Haematology (Wiley) 113 (1): 49–51. doi:10.1046/j.1365-2141.2001.02682.x. ISSN 0007-1048. PMID 11328280. 
  9. ↑ Yoshida, Masumi; Koshiyama, Masafumi; Fujii, Haruko; Konishi, Mitsunaga (1999). "Erythrocytosis and a fibroid". The Lancet (Elsevier BV) 354 (9174): 216. doi:10.1016/s0140-6736(99)02273-4. ISSN 0140-6736. PMID 10421304. 
  10. ↑ Fleming, Arthur R.; Markley, John C. (1957). "Polycythemia associated with uterine myomas". American Journal of Obstetrics and Gynecology (Elsevier BV) 74 (3): 677–679. doi:10.1016/0002-9378(57)90528-8. ISSN 0002-9378. PMID 13458274. 
  11. ↑ Wrigley, P. F. M.; Malpas, J. S.; Turnbull, A. L.; Jenkins, G. C.; McArt, A. (1971). "Secondary Polycythaemia due to a Uterine Fibromyoma Producing Erythropoietin". British Journal of Haematology 21 (5): 551–555. doi:10.1111/j.1365-2141.1971.tb02716.x. ISSN 0007-1048. PMID 5111704. 
  12. ↑ Maresh, M.J.A.; Metcalfe, M.A.; McPherson, Klim; Overton, C.; Hall, V.; Hargreaves, J.; Bridgman, S.; Dobbins, J. et al. (2002). "The VALUE national hysterectomy study: description of the patients and their surgery". BJOG: An International Journal of Obstetrics & Gynaecology (Wiley) 109 (3): 302–312. doi:10.1111/j.1471-0528.2002.01282.x. ISSN 1470-0328. PMID 11950186. 

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