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.