Titlebook: Cytokine Storm Syndrome; Randy Q. Cron,Edward M. Behrens Book 2024Latest edition The Editor(s) (if applicable) and The Author(s), under ex

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https://doi.org/10.1007/978-3-030-42882-2LH (henceforth referred to as “pHLH”) and are the focus of this chapter. Conversely, secondary HLH (sHLH) often occurs in the absence of monogenic etiologies that are commonly associated with pHLH and can be triggered by infections, malignancies, or rheumatological diseases; these triggers and the g

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https://doi.org/10.1007/978-3-030-42882-2sible for the frequently fatal multiorgan system failure seen in MAS. Whole exome sequencing as well as targeted sequencing of pHLH-associated genes in patients with SJIA-associated MAS demonstrated increased “burden” of rare protein-altering variants affecting the cytolytic pathway compared to heal

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History of Hemophagocytic Lymphohistiocytosiseceiving increased attention. The history of HLH dates back to 1939 when it was first described in adults, to be followed in 1952 by the first description of its primary, familial form in children. Secondary forms of HLH are far more frequent and occur with infections, malignancies, metabolic diseas

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