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
|