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Cytokine Storm Syndromes, including HLH and MAS, are frequently
fatal disorders, particularly if not recognized early and treated
during presentation. The genetics of Cytokine Storm Syndromes are
being defined with many of the risk alleles giving rise to
mutations in the perforin-mediated cytolytic pathway used by CD8
cytotoxic T cells and natural killer cells. These are being studied
using murine models. Up to 10% of the general population may carry
risk alleles for developing Cytokine Storm Syndromes, and Cytokine
Storm Syndromes are being increasingly recognized around the world
in pediatric and adult hospitals. A variety of infectious,
rheumatic, and oncologic triggers are commonly associated with
Cytokine Storm Syndromes, but understanding this disorder is
critical for all researchers and physicians to ensure timely and
appropriate therapy. This textbook, the first of its kind,
addresses all aspects of the disorder - from genetics,
pathophysiology, and ongoing research, to clinical presentations,
risk factors, and treatment.
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