Cytokine Storm Syndrome

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.

• Hemophagocytic lymphohistiocytosis
• Macrophage activation syndrome
• Perforin
• Cytolysis
• Genetics
• Cytokine Storm
• cytogenetics
• infectious diseases

• UAB School of Medicine, University of Alabama at Birmingham, Birmingham, USA

  Randy Q. Cron

• Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA

  Edward M. Behrens

Randy Q. Cron, MD, PhD                                              

University of Alabama at Birmingham

Professor of Pediatrics and Medicine

Birmingham

Alabama

USA

Edward M. Behrens, MD                 

University of Pennsylvania Perelman School of Medicine

Perelman School of Medicine

Philadelphia

Pennsylvania

USA

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