Myomatous erythrocytosis syndrome explained
| Myomatous erythrocytosis syndrome |
| Synonyms: | MES |
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
Further reading
- Suresh . Pooja . Rizk . Sanaa . Myomatous Erythrocytosis Syndrome: Case Report and Review of the Literature . Cureus . Cureus, Inc. . February 5, 2020 . 12 . 2 . e6892 . 2168-8184 . 10.7759/cureus.6892 . none . free. 32190455 . 7058389 .
- Shu . Xin-Yu . Chen . Na . Chen . Bi-Yun . Yang . Hui-Xia . Bi . Hui . Myomatous erythrocytosis syndrome: A case report . World Journal of Clinical Cases . Baishideng Publishing Group Inc. . 10 . 10 . April 6, 2022 . 2307-8960 . 10.12998/wjcc.v10.i10.3206 . 3206–3212 . 35611135 . none . free. 9082685 .
Notes and References
- Salaroli . Adriano . Couillandre . Pauline . Lewalle . Philippe . Benghiat . Samantha Fleur . Surprising erythrocytosis resolution after myomectomy: myomatous erythrocytosis syndrome . European Journal of Case Reports in Internal Medicine . SMC Media . March 18, 2023 . 10 . 3 . 2284-2594 . 10.12890/2023_003722 . 003722. free . 36969520 . 10035613 .
- Thomson . A.P. . Marson . F.G.W. . POLYCYTHÆMIA WITH FIBROIDS . The Lancet . Elsevier BV . 262 . 6789 . 1953 . 0140-6736 . 10.1016/s0140-6736(53)91456-1 . 759–760.
- Mui . Justin . Yang . Michael M.H. . Cohen . Trevor . McDonald . David I. . Hunt . Harold . More Than a Myoma: A Review of Myomatous Erythrocytosis Syndrome . Journal of Obstetrics and Gynaecology Canada . Elsevier BV . 42 . 2 . 2020 . 1701-2163 . 10.1016/j.jogc.2018.12.025 . 198–203.e3. 30904341 . 85496723 .
- Maudsley . R. F. . Boehm . F. H. . Secondary polycythemia and uterine leiomyomata. . Canadian Medical Association Journal . Canadian Medical Association . 103 . 5 . September 9, 1970 . 517–518 . 5469625 . 1930475 .
- Horwitz . Alec . Polycythemia Associated with Uterine Myomas . Journal of the American Medical Association . American Medical Association (AMA) . 158 . 15 . August 13, 1955 . 1360–1361 . 0002-9955 . 10.1001/jama.1955.02960150030009 . 13242355 .
- Paranjothy . D. . Vaish . S. K. . Polycythaemia Associated with Leiomyoma of the Uterus . BJOG: An International Journal of Obstetrics & Gynaecology . 74 . 4 . 1967 . 1470-0328 . 10.1111/j.1471-0528.1967.tb04001.x . 603–605. 6033281 . 42032143 .
- LEVGUR . M . DLEVIE . M . The myomatous erythrocytosis syndrome: A review . Obstetrics & Gynecology . Ovid Technologies (Wolters Kluwer Health) . 86 . 6 . 1995 . 0029-7844 . 10.1016/0029-7844(95)00292-y . 1026–1030. 7501327 . 25833390 .
- Suzuki . Mitsuaki . Takamizawa . Satoru . Nomaguchi . Koji . Suzu . Shinya . Yamada . Muneo . Igarashi . Takashi . Sato . Ikuo . Erythropoietin synthesis by tumour tissues in a patient with uterine myoma and erythrocytosis . British Journal of Haematology . Wiley . 113 . 1 . 2001 . 0007-1048 . 10.1046/j.1365-2141.2001.02682.x . 49–51. 11328280 . free .
- Yoshida . Masumi . Koshiyama . Masafumi . Fujii . Haruko . Konishi . Mitsunaga . Erythrocytosis and a fibroid . The Lancet . Elsevier BV . 354 . 9174 . 1999 . 0140-6736 . 10.1016/s0140-6736(99)02273-4 . 216. 10421304 . 42615650 .
- Fleming . Arthur R. . Markley . John C. . Polycythemia associated with uterine myomas . American Journal of Obstetrics and Gynecology . Elsevier BV . 74 . 3 . 1957 . 0002-9378 . 10.1016/0002-9378(57)90528-8 . 677–679. 13458274 .
- Wrigley . P. F. M. . Malpas . J. S. . Turnbull . A. L. . Jenkins . G. C. . McArt . A. . Secondary Polycythaemia due to a Uterine Fibromyoma Producing Erythropoietin . British Journal of Haematology . 21 . 5 . 1971 . 0007-1048 . 10.1111/j.1365-2141.1971.tb02716.x . 551–555. 5111704 . 20819568 .
- Maresh . M.J.A. . Metcalfe . M.A. . McPherson . Klim . Overton . C. . Hall . V. . Hargreaves . J. . Bridgman . S. . Dobbins . J. . Casbard . A. . The VALUE national hysterectomy study: description of the patients and their surgery . BJOG: An International Journal of Obstetrics & Gynaecology . Wiley . 109 . 3 . 2002 . 1470-0328 . 10.1111/j.1471-0528.2002.01282.x . 302–312. 11950186 . free .
