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Mast cell activation syndrome

MedGen UID:
1698540
Concept ID:
C5200989
Disease or Syndrome
Synonyms: Idiopathic Mast Cell Activation Disorder; Mast Cell Activation Syndrome; Mast Cell Activation Syndromes; nc MCAS; nc-MCAS; Non Clonal Mast Cell Activation Disorder; Non Clonal Mast Cell Activation Syndrome; Non-Clonal Mast Cell Activation Disorder; Non-Clonal Mast Cell Activation Syndrome; Nonclonal Mast Cell Activation Syndrome
SNOMED CT: Mast cell activation syndrome (16697871000119100); MCAD - mast cell activation disorder (16697871000119100)
 
Monarch Initiative: MONDO:0100004

Definition

A clinically defined disease states with a largely unknown morphological background. Acute mast cell activation (MCA) is commonly seen in allergic reactions and often leads to the clinical signs and symptoms of anaphylaxis. Severe or even life-threatening MCA may occur when the burden of mast cells is high and/or these cells are in an hyperactivated state. Mastocytosis may be associated with mast cell activation syndrome (MCAS). [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMast cell activation syndrome

Professional guidelines

PubMed

Valent P, Akin C, Nedoszytko B, Bonadonna P, Hartmann K, Niedoszytko M, Brockow K, Siebenhaar F, Triggiani M, Arock M, Romantowski J, Górska A, Schwartz LB, Metcalfe DD
Int J Mol Sci 2020 Nov 27;21(23) doi: 10.3390/ijms21239030. PMID: 33261124Free PMC Article
Weiler CR
J Allergy Clin Immunol Pract 2020 Feb;8(2):498-506. Epub 2019 Aug 27 doi: 10.1016/j.jaip.2019.08.022. PMID: 31470118
Castells M, Butterfield J
J Allergy Clin Immunol Pract 2019 Apr;7(4):1097-1106. doi: 10.1016/j.jaip.2019.02.002. PMID: 30961835

Recent clinical studies

Etiology

Bonadonna P, Nalin F, Olivieri F
Curr Opin Allergy Clin Immunol 2022 Oct 1;22(5):277-282. Epub 2022 Aug 4 doi: 10.1097/ACI.0000000000000849. PMID: 35942852
Valent P, Hartmann K, Bonadonna P, Niedoszytko M, Triggiani M, Arock M, Brockow K
Int Arch Allergy Immunol 2022;183(7):693-705. Epub 2022 May 23 doi: 10.1159/000524532. PMID: 35605594Free PMC Article
Yong SJ
Infect Dis (Lond) 2021 Oct;53(10):737-754. Epub 2021 May 22 doi: 10.1080/23744235.2021.1924397. PMID: 34024217Free PMC Article
Dorff SR, Afrin LB
J Obstet Gynaecol 2020 Oct;40(7):889-901. Epub 2020 Mar 9 doi: 10.1080/01443615.2019.1674259. PMID: 32148151
Weiler CR
J Allergy Clin Immunol Pract 2020 Feb;8(2):498-506. Epub 2019 Aug 27 doi: 10.1016/j.jaip.2019.08.022. PMID: 31470118

Diagnosis

Matito A, Escribese MM, Longo N, Mayorga C, Luengo-Sánchez O, Pérez-Gordo M, Matheu V, Labrador-Horrillo M, Pascal M, Seoane-Reula ME; Comité de Inmunología de la Sociedad Española de Alergología e Inmunología Clínica (SEAIC)
J Investig Allergol Clin Immunol 2021 Dec 21;31(6):461-470. Epub 2020 Feb 5 doi: 10.18176/jiaci.0675. PMID: 33541851
Weinstock LB, Pace LA, Rezaie A, Afrin LB, Molderings GJ
Dig Dis Sci 2021 Apr;66(4):965-982. Epub 2020 Apr 23 doi: 10.1007/s10620-020-06264-9. PMID: 32328892
Afrin LB, Ackerley MB, Bluestein LS, Brewer JH, Brook JB, Buchanan AD, Cuni JR, Davey WP, Dempsey TT, Dorff SR, Dubravec MS, Guggenheim AG, Hindman KJ, Hoffman B, Kaufman DL, Kratzer SJ, Lee TM, Marantz MS, Maxwell AJ, McCann KK, McKee DL, Menk Otto L, Pace LA, Perkins DD, Radovsky L, Raleigh MS, Rapaport SA, Reinhold EJ, Renneker ML, Robinson WA, Roland AM, Rosenbloom ES, Rowe PC, Ruhoy IS, Saperstein DS, Schlosser DA, Schofield JR, Settle JE, Weinstock LB, Wengenroth M, Westaway M, Xi SC, Molderings GJ
Diagnosis (Berl) 2021 May 26;8(2):137-152. Epub 2020 Apr 22 doi: 10.1515/dx-2020-0005. PMID: 32324159
Weiler CR
J Allergy Clin Immunol Pract 2020 Feb;8(2):498-506. Epub 2019 Aug 27 doi: 10.1016/j.jaip.2019.08.022. PMID: 31470118
Frieri M
Clin Rev Allergy Immunol 2018 Jun;54(3):353-365. doi: 10.1007/s12016-015-8487-6. PMID: 25944644

Therapy

Theoharides TC, Perlman AI, Twahir A, Kempuraj D
Expert Rev Clin Immunol 2023 Jun;19(6):639-654. Epub 2023 Apr 14 doi: 10.1080/1744666X.2023.2200936. PMID: 37029958
Theoharides TC, Cholevas C, Polyzoidis K, Politis A
Biofactors 2021 Mar;47(2):232-241. Epub 2021 Apr 12 doi: 10.1002/biof.1726. PMID: 33847020Free PMC Article
Theoharides TC, Kavalioti M, Martinotti R
J Biol Regul Homeost Agents 2019 Nov-Dec;33(6):1663-1667. doi: 10.23812/19-33n6Edit_Theoharides. PMID: 31928596
Castells M, Butterfield J
J Allergy Clin Immunol Pract 2019 Apr;7(4):1097-1106. doi: 10.1016/j.jaip.2019.02.002. PMID: 30961835
Frieri M
Clin Rev Allergy Immunol 2018 Jun;54(3):353-365. doi: 10.1007/s12016-015-8487-6. PMID: 25944644

Prognosis

Batiha GE, Al-Kuraishy HM, Al-Gareeb AI, Welson NN
Virol J 2022 Oct 9;19(1):158. doi: 10.1186/s12985-022-01891-2. PMID: 36210445Free PMC Article
Matito A, Escribese MM, Longo N, Mayorga C, Luengo-Sánchez O, Pérez-Gordo M, Matheu V, Labrador-Horrillo M, Pascal M, Seoane-Reula ME; Comité de Inmunología de la Sociedad Española de Alergología e Inmunología Clínica (SEAIC)
J Investig Allergol Clin Immunol 2021 Dec 21;31(6):461-470. Epub 2020 Feb 5 doi: 10.18176/jiaci.0675. PMID: 33541851
Klaiber N, Kumar S, Irani AM
Curr Allergy Asthma Rep 2017 Oct 7;17(11):80. doi: 10.1007/s11882-017-0748-4. PMID: 28988349
Gülen T, Akin C
Curr Opin Allergy Clin Immunol 2017 Aug;17(4):295-303. doi: 10.1097/ACI.0000000000000377. PMID: 28570344
Fenny N, Grammer LC
Immunol Allergy Clin North Am 2015 May;35(2):349-62. Epub 2015 Mar 6 doi: 10.1016/j.iac.2015.01.004. PMID: 25841556

Clinical prediction guides

González de Olano D, Cain WV, Bernstein JA, Akin C
J Allergy Clin Immunol Pract 2023 Jul;11(7):1989-1996. Epub 2023 May 21 doi: 10.1016/j.jaip.2023.05.012. PMID: 37220812
Batiha GE, Al-Kuraishy HM, Al-Gareeb AI, Welson NN
Virol J 2022 Oct 9;19(1):158. doi: 10.1186/s12985-022-01891-2. PMID: 36210445Free PMC Article
Szukiewicz D, Wojdasiewicz P, Watroba M, Szewczyk G
J Immunol Res 2022;2022:9534163. Epub 2022 Jun 22 doi: 10.1155/2022/9534163. PMID: 35785029Free PMC Article
Matito A, Escribese MM, Longo N, Mayorga C, Luengo-Sánchez O, Pérez-Gordo M, Matheu V, Labrador-Horrillo M, Pascal M, Seoane-Reula ME; Comité de Inmunología de la Sociedad Española de Alergología e Inmunología Clínica (SEAIC)
J Investig Allergol Clin Immunol 2021 Dec 21;31(6):461-470. Epub 2020 Feb 5 doi: 10.18176/jiaci.0675. PMID: 33541851
Gülen T, Akin C
Curr Opin Allergy Clin Immunol 2017 Aug;17(4):295-303. doi: 10.1097/ACI.0000000000000377. PMID: 28570344

Recent systematic reviews

Sabato V, Michel M, Blank U, Ebo DG, Vitte J
Curr Opin Allergy Clin Immunol 2021 Oct 1;21(5):426-434. doi: 10.1097/ACI.0000000000000768. PMID: 34292177
Nurmatov UB, Rhatigan E, Simons FE, Sheikh A
Allergy 2015 Sep;70(9):1052-61. Epub 2015 Jul 6 doi: 10.1111/all.12672. PMID: 26095756

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