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.

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.