© 2019

Cytokine Storm Syndrome

  • Randy Q. Cron
  • Edward M. Behrens

Table of contents

  1. Front Matter
    Pages i-xxi
  2. Background

    1. Front Matter
      Pages 1-1
    2. Flavia G. Rosado, Purva Gopal
      Pages 43-59
    3. Francesca Minoia, Sergio Davì, Alessandra Alongi, Angelo Ravelli
      Pages 61-79
  3. Genetics of Cytokine Storm Syndromes

    1. Front Matter
      Pages 81-81
    2. Spyridon Karageorgos, Hamid Bassiri
      Pages 83-111
    3. Grant S. Schulert, Kejian Zhang
      Pages 113-129
  4. Immunology of Cytokine Storm Syndromes

    1. Front Matter
      Pages 139-139
    2. Takuya Sekine, Donatella Galgano, Giovanna P. Casoni, Marie Meeths, Yenan T. Bryceson
      Pages 141-161
    3. Anthony R. French, Megan A. Cooper
      Pages 163-181
    4. Edward M. Behrens
      Pages 197-207
    5. David A. Hill, Neil Romberg
      Pages 209-232
  5. Infectious Triggers of Cytokine Storm Syndromes

    1. Front Matter
      Pages 233-233
    2. Katherine C. Verbist, Kim E. Nichols
      Pages 253-276

About this book


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

Editors and affiliations

  • Randy Q. Cron
    • 1
  • Edward M. Behrens
    • 2
  1. 1.UAB School of MedicineUniversity of Alabama at BirminghamBirminghamUSA
  2. 2.Perelman School of MedicineUniversity of PennsylvaniaPhiladelphiaUSA

About the editors

Randy Q. Cron, MD, PhD                                              

University of Alabama at Birmingham

Professor of Pediatrics and Medicine




Edward M. Behrens, MD                 

University of Pennsylvania Perelman School of Medicine

Perelman School of Medicine




Bibliographic information