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Plethora

MedGen UID:
115911
Concept ID:
C0232370
Finding
Synonym: Florid red complexion
SNOMED CT: Plethora (75246004); Florid red complexion (75246004)
 
HPO: HP:0001050

Conditions with this feature

Chuvash polycythemia
MedGen UID:
332974
Concept ID:
C1837915
Disease or Syndrome
Familial erythrocytosis-2 (ECYT2) is an autosomal recessive disorder characterized by increased red blood cell mass, increased serum levels of erythropoietin (EPO; 133170), and normal oxygen affinity. Patients with ECYT2 carry a high risk for peripheral thrombosis and cerebrovascular events (Cario, 2005). Familial erythrocytosis-2 has features of both primary and secondary erythrocytosis. In addition to increased circulating levels of EPO, consistent with a secondary, extrinsic process, erythroid progenitors may be hypersensitive to EPO, consistent with a primary, intrinsic process (Prchal, 2005). For a general phenotypic description and a discussion of genetic heterogeneity of familial erythrocytosis, see ECYT1 (133100).
Primary familial polycythemia due to EPO receptor mutation
MedGen UID:
1641215
Concept ID:
C4551637
Disease or Syndrome
Primary familial and congenital polycythemia (PFCP) is characterized by isolated erythrocytosis in an individual with a normal-sized spleen and absence of disorders causing secondary erythrocytosis. Clinical manifestations relate to the erythrocytosis and can include plethora, the hyperviscosity syndrome (headache, dizziness, fatigue, lassitude, visual and auditory disturbances, paresthesia, myalgia), altered mental status caused by hypoperfusion and local hypoxia, and arterial and/or venous thromboembolic events. Although the majority of individuals with PFCP have only mild manifestations of hyperviscosity such as dizziness or headache, some affected individuals have had severe and even fatal complications including arterial hypertension, intracerebral hemorrhage, deep vein thrombosis, coronary disease, and myocardial infarction. To date 116 affected individuals from 24 families have been reported.

Professional guidelines

PubMed

Alexander M, Kim SY, Cheng H
Lung 2020 Dec;198(6):897-907. Epub 2020 Nov 11 doi: 10.1007/s00408-020-00407-5. PMID: 33175991Free PMC Article
Modi AR, Kovacs CS
Cleve Clin J Med 2020 Oct 1;87(10):633-639. doi: 10.3949/ccjm.87a.19117. PMID: 33004324
Popovic D, Vucic D, Dikic I
Nat Med 2014 Nov;20(11):1242-53. Epub 2014 Nov 6 doi: 10.1038/nm.3739. PMID: 25375928

Recent clinical studies

Etiology

Grütz K, Klein C
J Neural Transm (Vienna) 2021 Apr;128(4):395-404. Epub 2021 Feb 19 doi: 10.1007/s00702-021-02314-2. PMID: 33604773Free PMC Article
Ben-Aicha S, Badimon L, Vilahur G
Int J Mol Sci 2020 Jan 22;21(3) doi: 10.3390/ijms21030732. PMID: 31979129Free PMC Article
Vakamudi S, Ho N, Cremer PC
Prog Cardiovasc Dis 2017 Jan-Feb;59(4):380-388. Epub 2017 Jan 4 doi: 10.1016/j.pcad.2016.12.009. PMID: 28062268
Edward KL, Ousey K, Warelow P, Lui S
Br J Nurs 2014 Jun 26-Jul 9;23(12):653-4, 656-9. doi: 10.12968/bjon.2014.23.12.653. PMID: 25039630
Munro MG, Critchley HO, Broder MS, Fraser IS; FIGO Working Group on Menstrual Disorders
Int J Gynaecol Obstet 2011 Apr;113(1):3-13. Epub 2011 Feb 22 doi: 10.1016/j.ijgo.2010.11.011. PMID: 21345435

Diagnosis

Grütz K, Klein C
J Neural Transm (Vienna) 2021 Apr;128(4):395-404. Epub 2021 Feb 19 doi: 10.1007/s00702-021-02314-2. PMID: 33604773Free PMC Article
McCombie WR, McPherson JD, Mardis ER
Cold Spring Harb Perspect Med 2019 Nov 1;9(11) doi: 10.1101/cshperspect.a036798. PMID: 30478097Free PMC Article
Vakamudi S, Ho N, Cremer PC
Prog Cardiovasc Dis 2017 Jan-Feb;59(4):380-388. Epub 2017 Jan 4 doi: 10.1016/j.pcad.2016.12.009. PMID: 28062268
Alshahrani F, Aljohani N
Nutrients 2013 Sep 13;5(9):3605-16. doi: 10.3390/nu5093605. PMID: 24067388Free PMC Article
Munro MG, Critchley HO, Broder MS, Fraser IS; FIGO Working Group on Menstrual Disorders
Int J Gynaecol Obstet 2011 Apr;113(1):3-13. Epub 2011 Feb 22 doi: 10.1016/j.ijgo.2010.11.011. PMID: 21345435

Therapy

Müller S, Bieber T, Ständer S
Expert Opin Biol Ther 2022 Jan;22(1):47-58. Epub 2021 Aug 4 doi: 10.1080/14712598.2021.1958777. PMID: 34289753
De Gregorio D, Aguilar-Valles A, Preller KH, Heifets BD, Hibicke M, Mitchell J, Gobbi G
J Neurosci 2021 Feb 3;41(5):891-900. Epub 2020 Nov 30 doi: 10.1523/JNEUROSCI.1659-20.2020. PMID: 33257322Free PMC Article
Tsimberidou AM, Fountzilas E, Nikanjam M, Kurzrock R
Cancer Treat Rev 2020 Jun;86:102019. Epub 2020 Mar 31 doi: 10.1016/j.ctrv.2020.102019. PMID: 32251926Free PMC Article
Pardoll DM
Nat Rev Cancer 2012 Mar 22;12(4):252-64. doi: 10.1038/nrc3239. PMID: 22437870Free PMC Article
Bernard GR, Artigas A, Brigham KL, Carlet J, Falke K, Hudson L, Lamy M, Legall JR, Morris A, Spragg R
Am J Respir Crit Care Med 1994 Mar;149(3 Pt 1):818-24. doi: 10.1164/ajrccm.149.3.7509706. PMID: 7509706

Prognosis

Nolan E, Lindeman GJ, Visvader JE
Cell 2023 Apr 13;186(8):1708-1728. Epub 2023 Mar 16 doi: 10.1016/j.cell.2023.01.040. PMID: 36931265
Gallo Marin B, Aghagoli G, Lavine K, Yang L, Siff EJ, Chiang SS, Salazar-Mather TP, Dumenco L, Savaria MC, Aung SN, Flanigan T, Michelow IC
Rev Med Virol 2021 Jan;31(1):1-10. Epub 2020 Jul 30 doi: 10.1002/rmv.2146. PMID: 32845042Free PMC Article
Barber TM, Kabisch S, Pfeiffer AFH, Weickert MO
Nutrients 2020 Oct 21;12(10) doi: 10.3390/nu12103209. PMID: 33096647Free PMC Article
Tsimberidou AM, Fountzilas E, Nikanjam M, Kurzrock R
Cancer Treat Rev 2020 Jun;86:102019. Epub 2020 Mar 31 doi: 10.1016/j.ctrv.2020.102019. PMID: 32251926Free PMC Article
Bernard GR, Artigas A, Brigham KL, Carlet J, Falke K, Hudson L, Lamy M, Legall JR, Morris A, Spragg R
Am J Respir Crit Care Med 1994 Mar;149(3 Pt 1):818-24. doi: 10.1164/ajrccm.149.3.7509706. PMID: 7509706

Clinical prediction guides

Usmani S, Saboor A, Haris M, Khan MA, Park H
Sensors (Basel) 2021 Jul 29;21(15) doi: 10.3390/s21155134. PMID: 34372371Free PMC Article
Grgic J
Sports Med 2021 Nov;51(11):2281-2298. Epub 2021 Jul 22 doi: 10.1007/s40279-021-01521-x. PMID: 34291426
Gangat N, Tefferi A
Br J Haematol 2020 Oct;191(2):152-170. Epub 2020 Mar 20 doi: 10.1111/bjh.16576. PMID: 32196650
Sharma A, Amarnath S, Thulasimani M, Ramaswamy S
Indian J Pharmacol 2016 May-Jun;48(3):237-40. doi: 10.4103/0253-7613.182888. PMID: 27298490Free PMC Article
Bowen WH
Odontology 2013 Jan;101(1):2-8. Epub 2012 Dec 6 doi: 10.1007/s10266-012-0092-z. PMID: 23224410

Recent systematic reviews

Androulakis-Korakakis P, Fisher JP, Steele J
Sports Med 2020 Apr;50(4):751-765. doi: 10.1007/s40279-019-01236-0. PMID: 31797219
Liu MY, Li N, Li WA, Khan H
Neurol Res 2017 Jun;39(6):573-580. Epub 2017 Apr 17 doi: 10.1080/01616412.2017.1317904. PMID: 28415916
Cooper K, Martyn-St James M, Kaltenthaler E, Dickinson K, Cantrell A
Health Technol Assess 2015 Mar;19(21):1-180, v-vi. doi: 10.3310/hta19210. PMID: 25768099Free PMC Article
Edward KL, Ousey K, Warelow P, Lui S
Br J Nurs 2014 Jun 26-Jul 9;23(12):653-4, 656-9. doi: 10.12968/bjon.2014.23.12.653. PMID: 25039630
Corrigan PW, Morris SB, Michaels PJ, Rafacz JD, Rüsch N
Psychiatr Serv 2012 Oct;63(10):963-73. doi: 10.1176/appi.ps.201100529. PMID: 23032675

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