Hyperferritinemic syndromes Hyperferritinemic syndromes Hyperferritinemic syndromes Hyperferritinemic syndromes
Name Aetiology Clinical features Therapeutic strategy
Secondary Hemophagocytic lymphohistiocytosis
Infections Viruses Bacteria Parasites Fungi Malignancies Mainly malignant lymphoma Autoinflammatory or autoimmune disorders Other causes Transplantation Metabolic Traumatic Iatrogenic (immunosuppression, vaccination, surgery, haemodialysis) Pregnancy
Fever, rash, hepatosplenomegaly, lymph node enlargement, bleeding diathesis, sepsis-like syndrome, variable degrees of neurologic symptoms, possibly rapidly unexpected progress to multiple organ failure
HLH-94 protocol: Glucocorticoids Cyclosporine A Intrathecal therapy Etoposide Treatment of the specific trigger/underlying disease: Glucocorticoids Anti-viral drugs Anti-CD20 (rituximab) Intravenous immunoglobulins Chemotherapy IL-1 inhibitors (anakinra, canakinumab) IL6 inhibitors (tocilizumab) Currently being tested: JAK1/2 inhibitors (ruxolitinib) anti–IFN-γ (alemtuzumab, emapalumab)
Catastrophic antiphospholipid syndrome
Trigger supposed to be infections in the presence of antiphospholipid antibodies
Microvascular thrombosis: renal insufficiency, acute respiratory distress syndrome/pulmonary embolism, encephalopathy, stroke, seizures, headache and coma, heart failure, myocardial infarction, valvular defects, livedo reticularis, skin necrosis and digital ischemia; spleen, adrenal glands, pancreas, retina and bone marrow infarction Intravenous heparin
Glucocorticoids
Intravenous immunoglobulins
Cyclophosphamide
Anti-CD20 (rituximab)
Plasmapheresis
Eculizumab
Still’s disease
Not clearly defined Viruses Bacteria Solid cancers Haematological malignancies Fever, arthritis, skin rash, myalgias, splenomegaly, lymphadenopathy, sore throat, liver involvement, pleurisy or pericarditis, abdominal pain, aseptic meningitis, disseminated intravascular coagulation, haemolysis Glucocorticoids
Hydroxychloroquine
Intravenous immunoglobulins
Methotrexate
Cyclosporine
IL-1 inhibitors (anakinra, canakinumab, rilonacept)
Anti-IL6 (tocilizumab)
TNF-inhibitors (infliximab, etanercept and adalimumab)
Septic shock
Infections Viruses Bacteria Parasites Fungi
Fever, rash, disseminated intravascular coagulation, variable degrees of neurologic symptoms, possibly rapidly unexpected progress to multiple organ failure
Broad spectrum antibiotic therapy
Fluid resuscitation
Inotropic drugs