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. 2022 Aug 19;1(3):37667.
doi: 10.56305/001c.37667. eCollection 2022.

Update in Hyperferritinemic Syndromes: Recognition and Management - A Scoping Review

Athanasios Vassilopoulos  1 , Winston McCormick  2 , Alisha Lakhani  3
Affiliations

Update in Hyperferritinemic Syndromes: Recognition and Management - A Scoping Review

Athanasios Vassilopoulos et al. Brown J Hosp Med. .

Abstract

Elevated serum ferritin is a marker of macrophage activation and is associated with increased mortality. The hyperferritinemic syndromes which include hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS), catastrophic antiphospholipid syndrome (CAPS), septic shock, adult-onset Still's Disease (AOSD), and multi-inflammatory syndrome related to COVID-19 (MIS-C/A) are characterized by intense inflammation and its sequalae. Prompt recognition and management of these heterogenous disorders is required to improve patient outcomes. We perform a scoping review of the existing literature on the key features of these rare syndromes.

Keywords: adult-onset Still’s Disease; catastrophic antiphospholipid syndrome; hemophagocytic lymphohistiocytosis; hyperferritinemic; macrophage activation syndrome; multi- inflammatory syndrome; septic shock.

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Conflict of interest statement

The authors have no conflicts of interest to disclose.

References

    1. The ferritins: molecular properties, iron storage function and cellular regulation. Harrison Pauline M., Arosio Paolo. Jul;1996 Biochimica et Biophysica Acta (BBA) - Bioenergetics. 1275(3):161–203. doi: 10.1016/0005-2728(96)00022-9. https://doi.org/10.1016/0005-2728(96)00022-9 - DOI - DOI - PubMed
    1. Detection of a glycosylated subunit in human serum ferritin. Cragg S J, Wagstaff M, Worwood M. Dec 1;1981 Biochemical Journal. 199(3):565–571. doi: 10.1042/bj1990565. https://doi.org/10.1042/bj1990565 - DOI - DOI - PMC - PubMed
    1. Percentage of glycosylated serum ferritin remains low throughout the course of adult onset Still's disease. Vignes S., Le Moël G., Fautrel B., Wechsler B., Godeau P., Piette J.C. May 1;2000 Annals of the Rheumatic Diseases. 59(5):347–350. doi: 10.1136/ard.59.5.347. https://doi.org/10.1136/ard.59.5.347 - DOI - DOI - PMC - PubMed
    1. High ferritin and low glycosylated ferritin may also be a marker of excessive macrophage activation. Lambotte O., Cacoub P., Costedoat N., Le Moel G., Amoura Z., Piette J.C. J Rheumatol. 30(5):1027–1028. - PubMed
    1. Weis Sebastian, Carlos Ana Rita, Moita Maria Raquel, Singh Sumnima, Blankenhaus Birte, Cardoso Silvia, Larsen Rasmus, Rebelo Sofia, Schäuble Sascha, Del Barrio Laura, Mithieux Gilles, Rajas Fabienne, Lindig Sandro, Bauer Michael, Soares Miguel P. Cell. 7. Vol. 169. Elsevier BV; Metabolic Adaptation Establishes Disease Tolerance to Sepsis; pp. 1263–1275.e14.https://doi.org/10.1016/j.cell.2017.05.031 - DOI - DOI - PMC - PubMed

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