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Macrophage activation syndrome

MedGen UID:
242753
Concept ID:
C1096155
Disease or Syndrome
Synonyms: Macrophage Activation Syndrome; Syndrome, Macrophage Activation
SNOMED CT: Macrophage activation syndrome (430478003); Hemophagocytic lymphohistiocytosis with rheumatologic disease (430478003); Reactive hemophagocytic syndrome (430478003)
 
Monarch Initiative: MONDO:0015545
Orphanet: ORPHA158061

Definition

A rare hemophagocytic syndrome characterized by excessive activation and proliferation of macrophages and T cells occurring in the context of a variety of diseases, including infections, neoplasms, rheumatic disorders, and leading to sudden onset of persistent fever, lymphadenopathy, and hepatosplenomegaly. Complications include profound depression of one or more blood cell lines with coagulopathy and pancytopenia, and impaired liver and renal function. Bone marrow examination reveals numerous well differentiated macrophages actively phagocytosing hematopoietic elements. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMacrophage activation syndrome
Follow this link to review classifications for Macrophage activation syndrome in Orphanet.

Professional guidelines

PubMed

Hines MR, von Bahr Greenwood T, Beutel G, Beutel K, Hays JA, Horne A, Janka G, Jordan MB, van Laar JAM, Lachmann G, Lehmberg K, Machowicz R, Miettunen P, La Rosée P, Shakoory B, Zinter MS, Henter JI
Crit Care Med 2022 May 1;50(5):860-872. Epub 2021 Oct 5 doi: 10.1097/CCM.0000000000005361. PMID: 34605776
Efthimiou P, Kontzias A, Hur P, Rodha K, Ramakrishna GS, Nakasato P
Semin Arthritis Rheum 2021 Aug;51(4):858-874. Epub 2021 Jun 13 doi: 10.1016/j.semarthrit.2021.06.004. PMID: 34175791
Henderson LA, Cron RQ
Paediatr Drugs 2020 Feb;22(1):29-44. doi: 10.1007/s40272-019-00367-1. PMID: 31732958Free PMC Article

Recent clinical studies

Etiology

Henderson LA, Cron RQ
Paediatr Drugs 2020 Feb;22(1):29-44. doi: 10.1007/s40272-019-00367-1. PMID: 31732958Free PMC Article
Skinner J, Yankey B, Shelton BK
AACN Adv Crit Care 2019 Summer;30(2):151-164. doi: 10.4037/aacnacc2019463. PMID: 31151946
Karakike E, Giamarellos-Bourboulis EJ
Front Immunol 2019;10:55. Epub 2019 Jan 31 doi: 10.3389/fimmu.2019.00055. PMID: 30766533Free PMC Article
Emile JF, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, Requena-Caballero L, Jordan MB, Abdel-Wahab O, Allen CE, Charlotte F, Diamond EL, Egeler RM, Fischer A, Herrera JG, Henter JI, Janku F, Merad M, Picarsic J, Rodriguez-Galindo C, Rollins BJ, Tazi A, Vassallo R, Weiss LM; Histiocyte Society
Blood 2016 Jun 2;127(22):2672-81. Epub 2016 Mar 10 doi: 10.1182/blood-2016-01-690636. PMID: 26966089Free PMC Article
Ravelli A, Davì S, Minoia F, Martini A, Cron RQ
Hematol Oncol Clin North Am 2015 Oct;29(5):927-41. Epub 2015 Aug 25 doi: 10.1016/j.hoc.2015.06.010. PMID: 26461152

Diagnosis

Cron RQ, Goyal G, Chatham WW
Annu Rev Med 2023 Jan 27;74:321-337. Epub 2022 Oct 13 doi: 10.1146/annurev-med-042921-112837. PMID: 36228171
Kellum JA, Formeck CL, Kernan KF, Gómez H, Carcillo JA
Crit Care Clin 2022 Apr;38(2):195-211. doi: 10.1016/j.ccc.2021.11.013. PMID: 35369943
Griffin G, Shenoi S, Hughes GC
Best Pract Res Clin Rheumatol 2020 Aug;34(4):101515. Epub 2020 May 7 doi: 10.1016/j.berh.2020.101515. PMID: 32387063
Henderson LA, Cron RQ
Paediatr Drugs 2020 Feb;22(1):29-44. doi: 10.1007/s40272-019-00367-1. PMID: 31732958Free PMC Article
Ravelli A, Davì S, Minoia F, Martini A, Cron RQ
Hematol Oncol Clin North Am 2015 Oct;29(5):927-41. Epub 2015 Aug 25 doi: 10.1016/j.hoc.2015.06.010. PMID: 26461152

Therapy

Fanouriakis A, Tziolos N, Bertsias G, Boumpas DT
Ann Rheum Dis 2021 Jan;80(1):14-25. Epub 2020 Oct 13 doi: 10.1136/annrheumdis-2020-218272. PMID: 33051219
Hanff TC, Mohareb AM, Giri J, Cohen JB, Chirinos JA
Am J Hematol 2020 Dec;95(12):1578-1589. Epub 2020 Sep 16 doi: 10.1002/ajh.25982. PMID: 32857878Free PMC Article
Giacomelli R, Ruscitti P, Shoenfeld Y
J Autoimmun 2018 Sep;93:24-36. Epub 2018 Aug 1 doi: 10.1016/j.jaut.2018.07.018. PMID: 30077425
Lee JJY, Schneider R
Pediatr Clin North Am 2018 Aug;65(4):691-709. doi: 10.1016/j.pcl.2018.04.005. PMID: 30031494
Barut K, Adrovic A, Şahin S, Kasapçopur Ö
Balkan Med J 2017 Apr 5;34(2):90-101. doi: 10.4274/balkanmedj.2017.0111. PMID: 28418334Free PMC Article

Prognosis

Fanouriakis A, Tziolos N, Bertsias G, Boumpas DT
Ann Rheum Dis 2021 Jan;80(1):14-25. Epub 2020 Oct 13 doi: 10.1136/annrheumdis-2020-218272. PMID: 33051219
Hanff TC, Mohareb AM, Giri J, Cohen JB, Chirinos JA
Am J Hematol 2020 Dec;95(12):1578-1589. Epub 2020 Sep 16 doi: 10.1002/ajh.25982. PMID: 32857878Free PMC Article
Giacomelli R, Ruscitti P, Shoenfeld Y
J Autoimmun 2018 Sep;93:24-36. Epub 2018 Aug 1 doi: 10.1016/j.jaut.2018.07.018. PMID: 30077425
Barut K, Adrovic A, Şahin S, Kasapçopur Ö
Balkan Med J 2017 Apr 5;34(2):90-101. doi: 10.4274/balkanmedj.2017.0111. PMID: 28418334Free PMC Article
Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P
Autoimmun Rev 2014 Jul;13(7):708-22. Epub 2014 Mar 19 doi: 10.1016/j.autrev.2014.01.058. PMID: 24657513

Clinical prediction guides

Efthimiou P, Kontzias A, Hur P, Rodha K, Ramakrishna GS, Nakasato P
Semin Arthritis Rheum 2021 Aug;51(4):858-874. Epub 2021 Jun 13 doi: 10.1016/j.semarthrit.2021.06.004. PMID: 34175791
Hanff TC, Mohareb AM, Giri J, Cohen JB, Chirinos JA
Am J Hematol 2020 Dec;95(12):1578-1589. Epub 2020 Sep 16 doi: 10.1002/ajh.25982. PMID: 32857878Free PMC Article
Karakike E, Giamarellos-Bourboulis EJ
Front Immunol 2019;10:55. Epub 2019 Jan 31 doi: 10.3389/fimmu.2019.00055. PMID: 30766533Free PMC Article
Giacomelli R, Ruscitti P, Shoenfeld Y
J Autoimmun 2018 Sep;93:24-36. Epub 2018 Aug 1 doi: 10.1016/j.jaut.2018.07.018. PMID: 30077425
Grom AA, Horne A, De Benedetti F
Nat Rev Rheumatol 2016 May;12(5):259-68. Epub 2016 Mar 24 doi: 10.1038/nrrheum.2015.179. PMID: 27009539Free PMC Article

Recent systematic reviews

Liu LL, Skribek M, Harmenberg U, Gerling M
J Immunother Cancer 2023 Mar;11(3) doi: 10.1136/jitc-2022-005841. PMID: 36878533Free PMC Article
Arnold DD, Yalamanoglu A, Boyman O
Front Immunol 2022;13:888392. Epub 2022 Jul 6 doi: 10.3389/fimmu.2022.888392. PMID: 35874710Free PMC Article
Onel KB, Horton DB, Lovell DJ, Shenoi S, Cuello CA, Angeles-Han ST, Becker ML, Cron RQ, Feldman BM, Ferguson PJ, Gewanter H, Guzman J, Kimura Y, Lee T, Murphy K, Nigrovic PA, Ombrello MJ, Rabinovich CE, Tesher M, Twilt M, Klein-Gitelman M, Barbar-Smiley F, Cooper AM, Edelheit B, Gillispie-Taylor M, Hays K, Mannion ML, Peterson R, Flanagan E, Saad N, Sullivan N, Szymanski AM, Trachtman R, Turgunbaev M, Veiga K, Turner AS, Reston JT
Arthritis Rheumatol 2022 Apr;74(4):553-569. Epub 2022 Mar 1 doi: 10.1002/art.42037. PMID: 35233993Free PMC Article
Onel KB, Horton DB, Lovell DJ, Shenoi S, Cuello CA, Angeles-Han ST, Becker ML, Cron RQ, Feldman BM, Ferguson PJ, Gewanter H, Guzman J, Kimura Y, Lee T, Murphy K, Nigrovic PA, Ombrello MJ, Rabinovich CE, Tesher M, Twilt M, Klein-Gitelman M, Barbar-Smiley F, Cooper AM, Edelheit B, Gillispie-Taylor M, Hays K, Mannion ML, Peterson R, Flanagan E, Saad N, Sullivan N, Szymanski AM, Trachtman R, Turgunbaev M, Veiga K, Turner AS, Reston JT
Arthritis Care Res (Hoboken) 2022 Apr;74(4):521-537. Epub 2022 Mar 1 doi: 10.1002/acr.24853. PMID: 35233986Free PMC Article
Poddighe D, Dauyey K
Rheumatol Int 2020 May;40(5):695-702. Epub 2019 Sep 16 doi: 10.1007/s00296-019-04442-1. PMID: 31529231

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