Subject(s)
Abdominal Pain/etiology , Adrenal Gland Diseases/pathology , Castleman Disease/diagnosis , Edema/pathology , Fever/etiology , Abdominal Pain/diagnosis , Antibodies, Monoclonal/administration & dosage , Antibodies, Monoclonal/therapeutic use , Antineoplastic Agents/administration & dosage , Antineoplastic Agents/therapeutic use , Antineoplastic Agents, Hormonal/administration & dosage , Antineoplastic Agents, Hormonal/therapeutic use , Azetidines/administration & dosage , Azetidines/therapeutic use , Bone Marrow/metabolism , Bone Marrow/pathology , Bortezomib/administration & dosage , Bortezomib/therapeutic use , Castleman Disease/drug therapy , Castleman Disease/pathology , Cyclosporine/administration & dosage , Cyclosporine/therapeutic use , Dexamethasone/administration & dosage , Dexamethasone/therapeutic use , Drug Therapy, Combination , Fever/diagnosis , Humans , Immunosuppressive Agents/administration & dosage , Immunosuppressive Agents/therapeutic use , Janus Kinase Inhibitors/administration & dosage , Janus Kinase Inhibitors/therapeutic use , Male , Middle Aged , Purines/administration & dosage , Purines/therapeutic use , Pyrazoles/administration & dosage , Pyrazoles/therapeutic use , Renal Insufficiency/etiology , Reticulin , Sulfonamides/administration & dosage , Sulfonamides/therapeutic use , Thrombocytopenia/etiologyABSTRACT
A subset of patients with severe COVID-19 develop profound inflammation and multi-organ dysfunction consistent with a "Cytokine Storm Syndrome" (CSS). In this review we compare the clinical features, diagnosis, and pathogenesis of COVID-CSS with other hematological CSS, namely secondary hemophagocytic lymphohistiocytosis (sHLH), idiopathic multicentric Castleman disease (iMCD), and CAR-T cell therapy associated Cytokine Release Syndrome (CRS). Novel therapeutics targeting cytokines or inhibiting cell signaling pathways have now become the mainstay of treatment in these CSS. We review the evidence for cytokine blockade and attenuation in these known CSS as well as the emerging literature and clinical trials pertaining to COVID-CSS. Established markers of inflammation as well as cytokine levels are compared and contrasted between these four entities in order to establish a foundation for future diagnostic criteria of COVID-CSS.