BookRandy Q. Cron, Edward M. Behrens, editors.
Summary: 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.
Contents:
History of Hemophagocytic Lymphohistiocytosis
The history of Macrophage Activation Syndrome in autoimmune diseases
Clinical features of Cytokine Storm Syndrome
Laboratory features and pathology of the Cytokine Storm Syndromes
Criteria for Cytokine Storm Syndromes
Genetics of primary Hemophagocytic Lymphohistiocytosis
Genetics of acquired Cytokine Storm Syndromes
Genetics of Macrophage Activation Syndrome in systemic juvenile idiopathic arthritis
CD8+ T cell biology in Cytokine Storm Syndromes
Immunology of Cytokine Storm Syndromes: natural killer cells
Myeloid cells in the immunopathogenesis of Cytokine Storm Syndromes
Cytokines in Cytokine Storm Syndrome
Primary immunodeficiencies and Cytokine Storm Syndromes
Infectious triggers of Cytokine Storm Syndromes: herpes virus family (non-EBV)
Cytokine Storm Syndromes associated with Epstein-Barr Virus
Cytokine Storm Syndrome associated with hemorrhagic fever and other viruses
Cytokine Storm Syndrome as a manifestation of primary HIV infection
Bacteria-associated Cytokine Storm Syndrome
Zoonotic bacterial infections triggering Cytokine Storm Syndrome
Parasitic and fungal triggers
Cytokine Storm Syndrome associated with systemic juvenile idiopathic arthritis
Systemic Lupus Erythematosus and cytokine storm
Kawasaki Disease-associated Cytokine Storm Syndrome
The intersections of autoinflammation and cytokine storm
Macrophage Activation Syndrome in the setting of rheumatic diseases
Hemophagocytic Lymphohistiocytosis in the context of hematological malignancies and solid tumors
Cytokine storm and sepsis-induced Multiple Organ Dysfunction Syndrome
Murine models of familial Cytokine Storm Syndromes
Murine models of secondary Cytokine Storm Syndromes
Etoposide therapy of Cytokine Storm Syndromes
IL-1 family blockade in Cytokine Storm Syndromes
IL-6 blockade in Cytokine Storm Syndromes
Anti-interferon-gamma therapy for Cytokine Storm Syndromes
Alternative therapies for Cytokine Storm Syndromes
Salvage therapy and allogeneic hematopoietic cell transplantation for the severe Cytokine Storm Syndrome of Hemophagocytic Lymphohistiocytosis.