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.

Provides the fundamental basic and applied clinical science at the cutting edge of CSS treatment First textbook devoted to Cytokine Storm Syndromes, covering all aspects of the disorder (clinical, genetics, pathophysiology, associations, therapy, and others) Extensive panel of contributing authors, made up of internationally-renowned experts in CSS research

Autorentext

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

Inhalt
Part I: Background.- 1. Cytokines in Cytokine Storm Syndrome.- 2. History of hemophagocytic lymphohistiocytosis.- 3.The History of Macrophage Activation Syndrome in Autoimmune Diseases.- 4. Clinical Features of Cytokine Storm Syndrome.- 5. Laboratory Features and Pathology of the Cytokine Storm Syndromes.- 6. Criteria for Cytokine Storm Syndromes.- Part II: Genetics of Cytokine Storm Syndromes.- 7.Genetics of Acquired Cytokine Storm Syndromes.- 8. Genetics of Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis.- Part III: Immunology of Cytokine Storm Syndromes.- 9. CD8+ T cell biology in cytokine storm syndromes.- 10. Immunology of Cytokine Storm Syndromes: Natural Killer Cells.- 11. Myeloid cells in the immunopathogenesis of cytokine storm syndromes.- 12. Cytokines in Cytokine Storm Syndrome.- 13. Primary Immunodeficiencies and Cytokine Storm Syndromes.- Part IV: Infectious Triggers of Cytokine Storm Syndromes.- 14. Infectious Triggers of Cytokine Storm Syndromes: Herpes Virus Family (non-EBV).- 15. Cytokine Storm Syndromes Associated with Epstein Barr Virus.- 16. Cytokine Storm Syndrome Associated with Hemorrhagic Fever and Other Viruses.- 17. Cytokine Storm Syndrome as a manifestation of Primary HIV Infection.- 18. Bacteria-Associated Cytokine Storm Syndrome.- 19. Zoonotic Bacterial Infections Triggering Cytokine Storm Syndrome.- 20. Parasitic and Fungal Triggers.- Part V: Rheumatic Triggers of Cytokine Storm Syndromes.- 21. Cytokine Storm Syndrome associated with Systemic Juvenile Idiopathic Arthritis.- 22. Systemic Lupus Erythematosus and Cytokine Storm.- 23. Kawasaki Disease.- 24. The Intersections of Autoinflammation and Cytokine Storm.- 25. Macrophage Activation Syndrome in the Setting of Rheumatic Diseases.- Part VI: Other Triggers of Cytokine Storm Syndromes.- 26. Hemophagocytic lymphohistiocytosis in the context of hematological malignancies and solid tumors.- 27. Cytokine Storm and Sepsis induced Multiple Organ Dysfunction Syndrome.- Part VII: Murine Models of Cytokine Storm Syndrom.- 28. Murine Models of Familial Cytokine Storm Syndromes.- 29. Murine Models of Secondary Cytokine Storm Syndromes.- Part VIII: Therapy of Cytokine Storm Syndromes.- 30. Etoposide Therapy of Cytokine Storm Syndromes.- 31. IL-1 Family Blockade.- 32. IL-6 Blockade.- 33. Alternative Therapies for Cytokine Storm Syndromes.- 34. Salvage Therapy and Allogeneic Hematopoietic Cell Transplantation for the Severe Cytokine Storm Syndrome of Hemophagocytic Lymphohistiocytosis.