View source for Medicine:Myomatous erythrocytosis syndrome

{{Infobox medical condition
 |name            = Myomatous erythrocytosis syndrome
 |synonyms        = MES
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'''Myomatous erythrocytosis syndrome''' '''(MES)''' is an uncommon [[Medicine:Gynaecology|gynecological disorder]] associated with isolated polycythemia and [[Medicine:Uterine fibroid|uterine fibroids]]. The primary feature of myomatous erythrocytosis syndrome is that [[Biology:Hemoglobin|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.<ref name="Surprising erythrocytosis">{{cite journal | last1=Salaroli | first1=Adriano | last2=Couillandre | first2=Pauline | last3=Lewalle | first3=Philippe | last4=Benghiat | first4=Samantha Fleur | title=Surprising erythrocytosis resolution after myomectomy: myomatous erythrocytosis syndrome | journal=European Journal of Case Reports in Internal Medicine | publisher=SMC Media | date=March 18, 2023 | volume=10 | issue=3 | issn=2284-2594 | doi=10.12890/2023_003722 | page=003722| doi-access=free | pmid=36969520 | pmc=10035613 }}</ref> Thomsen and Marson published the first case in 1953.<ref name="Thomson Marson">{{cite journal | last1=Thomson | first1=A.P. | last2=Marson | first2=F.G.W. | title=POLYCYTHÆMIA WITH FIBROIDS | journal=The Lancet | publisher=Elsevier BV | volume=262 | issue=6789 | year=1953 | issn=0140-6736 | doi=10.1016/s0140-6736(53)91456-1 | pages=759–760}}</ref>

== 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%).<ref name="More Than a Myoma">{{cite journal | last1=Mui | first1=Justin | last2=Yang | first2=Michael M.H. | last3=Cohen | first3=Trevor | last4=McDonald | first4=David I. | last5=Hunt | first5=Harold | title=More Than a Myoma: A Review of Myomatous Erythrocytosis Syndrome | journal=Journal of Obstetrics and Gynaecology Canada | publisher=Elsevier BV | volume=42 | issue=2 | year=2020 | issn=1701-2163 | doi=10.1016/j.jogc.2018.12.025 | pages=198–203.e3| pmid=30904341 | s2cid=85496723 }}</ref>

== Causes ==
Previous explanations for the etiology of myomatous erythrocytosis syndrome included the possibility of renal outflow obstruction,<ref name="Secondary polycythemia and uterine leiomyomata">{{cite journal | last1=Maudsley | first1=R. F. | last2=Boehm | first2=F. H. | title=Secondary polycythemia and uterine leiomyomata. | journal=Canadian Medical Association Journal | publisher=Canadian Medical Association | volume=103 | issue=5 | date=September 9, 1970 | pages=517–518 | pmid=5469625 | pmc=1930475 }}</ref> myomatous arteriovenous shunts,<ref name="Horwitz 1955 p. 1360">{{cite journal | last=Horwitz | first=Alec | title=Polycythemia Associated with Uterine Myomas | journal=Journal of the American Medical Association | publisher=American Medical Association (AMA) | volume=158 | issue=15 | date=August 13, 1955 | pages=1360–1361 | issn=0002-9955 | doi=10.1001/jama.1955.02960150030009 | pmid=13242355 }}</ref> or interference with pulmonary ventilation<ref name="Paranjothy Vaish 1967 pp. 603–605">{{cite journal | last1=Paranjothy | first1=D. | last2=Vaish | first2=S. K. | title=Polycythaemia Associated with Leiomyoma of the Uterus | journal=BJOG: An International Journal of Obstetrics & Gynaecology | volume=74 | issue=4 | date=1967 | issn=1470-0328 | doi=10.1111/j.1471-0528.1967.tb04001.x | pages=603–605| pmid=6033281 | s2cid=42032143 }}</ref> as possible pathways resulting in the hematological abnormalities that are characteristic of this illness. But these theories have been refuted.<ref name="LEVGUR DLEVIE 1995 pp. 1026–1030">{{cite journal | last1=LEVGUR | first1=M | last2=DLEVIE | first2=M | title=The myomatous erythrocytosis syndrome: A review | journal=Obstetrics & Gynecology | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=86 | issue=6 | year=1995 | issn=0029-7844 | doi=10.1016/0029-7844(95)00292-y | pages=1026–1030| pmid=7501327 | s2cid=25833390 }}</ref> The current theory regarding myomatous erythrocytosis syndrome links aberrant EPO production from the myomatous tissue to the observed hematological changes.<ref name="Suzuki Takamizawa Nomaguchi Suzu 2001 pp. 49–51">{{cite journal | last1=Suzuki | first1=Mitsuaki | last2=Takamizawa | first2=Satoru | last3=Nomaguchi | first3=Koji | last4=Suzu | first4=Shinya | last5=Yamada | first5=Muneo | last6=Igarashi | first6=Takashi | last7=Sato | first7=Ikuo | title=Erythropoietin synthesis by tumour tissues in a patient with uterine myoma and erythrocytosis | journal=British Journal of Haematology | publisher=Wiley | volume=113 | issue=1 | year=2001 | issn=0007-1048 | doi=10.1046/j.1365-2141.2001.02682.x | pages=49–51| pmid=11328280 | doi-access=free }}</ref><ref name="Yoshida Koshiyama Fujii Konishi 1999 p. 216">{{cite journal | last1=Yoshida | first1=Masumi | last2=Koshiyama | first2=Masafumi | last3=Fujii | first3=Haruko | last4=Konishi | first4=Mitsunaga | title=Erythrocytosis and a fibroid | journal=The Lancet | publisher=Elsevier BV | volume=354 | issue=9174 | year=1999 | issn=0140-6736 | doi=10.1016/s0140-6736(99)02273-4 | page=216| pmid=10421304 | s2cid=42615650 }}</ref>

== Diagnosis ==
Hematological values such as [[Biology:Hemoglobin|hemoglobin]], [[Medicine:Hematocrit|hematocrit]], and [[Biology:Red blood cell|red blood cell]] counts are elevated in patients with myomatous erythrocytosis syndrome.<ref name="More Than a Myoma"/> 

The diagnostic criteria for myomatous erythrocytosis syndrome includes polycythemia, uterine myoma, and return of [[Biology:Hemoglobin|hemoglobin]] levels to normal following fibroid excision surgery.<ref name="Fleming Markley 1957 pp. 677–679">{{cite journal | last1=Fleming | first1=Arthur R. | last2=Markley | first2=John C. | title=Polycythemia associated with uterine myomas | journal=American Journal of Obstetrics and Gynecology | publisher=Elsevier BV | volume=74 | issue=3 | year=1957 | issn=0002-9378 | doi=10.1016/0002-9378(57)90528-8 | pages=677–679| pmid=13458274 }}</ref>

== Treatment ==
[[Biology:Hysterectomy|Hysterectomy]] is the most common treatment for myomatous erythrocytosis syndrome.<ref name="More Than a Myoma"/> Horwitz and McKelway successfully performed the first myomectomy for myomatous erythrocytosis syndrome.<ref name="Wrigley Malpas Turnbull Jenkins 1971 pp. 551–555">{{cite journal | last1=Wrigley | first1=P. F. M. | last2=Malpas | first2=J. S. | last3=Turnbull | first3=A. L. | last4=Jenkins | first4=G. C. | last5=McArt | first5=A. | title=Secondary Polycythaemia due to a Uterine Fibromyoma Producing Erythropoietin | journal=British Journal of Haematology | volume=21 | issue=5 | date=1971 | issn=0007-1048 | doi=10.1111/j.1365-2141.1971.tb02716.x | pages=551–555| pmid=5111704 | s2cid=20819568 }}</ref> 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.<ref name="Maresh Metcalfe McPherson Overton 2002 pp. 302–312">{{cite journal | last1=Maresh | first1=M.J.A. | last2=Metcalfe | first2=M.A. | last3=McPherson | first3=Klim | last4=Overton | first4=C. | last5=Hall | first5=V. | last6=Hargreaves | first6=J. | last7=Bridgman | first7=S. | last8=Dobbins | first8=J. | last9=Casbard | first9=A. | title=The VALUE national hysterectomy study: description of the patients and their surgery | journal=BJOG: An International Journal of Obstetrics & Gynaecology | publisher=Wiley | volume=109 | issue=3 | year=2002 | issn=1470-0328 | doi=10.1111/j.1471-0528.2002.01282.x | pages=302–312| pmid=11950186 | doi-access=free }}</ref>

== See also ==
* [[Medicine:Uterine fibroid|Uterine fibroid]]
* [[Medicine:Polycythemia vera|Polycythemia vera]]

== References ==
{{reflist}}

== Further reading ==
* {{cite journal | last1=Suresh | first1=Pooja | last2=Rizk | first2=Sanaa | title=Myomatous Erythrocytosis Syndrome: Case Report and Review of the Literature | journal=Cureus | publisher=Cureus, Inc. | date=February 5, 2020 | volume=12 | issue=2 | pages=e6892 | issn=2168-8184 | doi=10.7759/cureus.6892 | ref=none | doi-access=free| pmid=32190455 | pmc=7058389 }}
* {{cite journal | last1=Shu | first1=Xin-Yu | last2=Chen | first2=Na | last3=Chen | first3=Bi-Yun | last4=Yang | first4=Hui-Xia | last5=Bi | first5=Hui | title=Myomatous erythrocytosis syndrome: A case report | journal=World Journal of Clinical Cases | publisher=Baishideng Publishing Group Inc. | volume=10 | issue=10 | date=April 6, 2022 | issn=2307-8960 | doi=10.12998/wjcc.v10.i10.3206 | pages=3206–3212 | pmid=35611135 | ref=none | doi-access=free| pmc=9082685 }}

== External links ==

{{Medical resources
| ICD11           = {{ICD11|3A81.Y}}
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[[Category:Syndromes]]

{{Sourceattribution|Myomatous erythrocytosis syndrome}}