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Cytokine Storm Syndrome pp 43–59Cite as

Laboratory Features and Pathology of the Cytokine Storm Syndromes

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Abstract

This chapter describes the proposed pathogenesis of the cytokine storm syndromes (hemophagocytic lymphohistiocytosis and macrophage activation syndrome), the laboratory findings associated with these diseases, and the currently recommended diagnostic strategies. The pathogenesis of primary forms of hemophagocytic lymphohistiocytosis (HLH) is based on an impaired function of cytotoxic T-lymphocytes and NK cells, which then leads to an inability of the immune system to clear the original antigenic insult, ultimately resulting in an uncontrolled release of cytokines. The exact pathogenesis of secondary HLH and macrophage activation syndrome, while presumably similar, is not well understood. Laboratory findings are nonspecific and comprise cytopenias, altered liver function tests, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia, altered cerebrospinal fluid analysis in patient with neurologic symptoms, and tissue hemophagocytosis. Diagnostic strategies include an initial testing assessment to detect the above-described changes that support the clinical concern for HLH. Disease-specific testing, available only in few laboratories, includes levels of soluble CD25, NK cell function assay, flow cytometric techniques for screening, and genetic testing for the mutations associated with primary HLH.

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Authors and Affiliations

  1. Department of Pathology, University of Texas Southwestern Biocenter, Dallas, TX, USA

    Flavia G. Rosado

  2. Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Purva Gopal

Authors
  1. Flavia G. Rosado
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  2. Purva Gopal
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Correspondence to Flavia G. Rosado .

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Editors and Affiliations

  1. UAB School of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA

    Randy Q. Cron

  2. Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Edward M. Behrens

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Rosado, F.G., Gopal, P. (2019). Laboratory Features and Pathology of the Cytokine Storm Syndromes. In: Cron, R., Behrens, E. (eds) Cytokine Storm Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-22094-5_4

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