Macrophage Activation/HLH Flashcards
HLH is a multi-system disorder characterized by dysregulated ____ and ___-cytokinemia
- immune system
- hyper
is HLH a disease entity?
what is it?
- no
- a clinical syndrome of hyper-inflammation
cause of HLH
- failure of CTLs/NK to kill infected cells
- uncontrolled expansion of CTLs because signal is still being sent
- uncontrolled activation of macrophages
diagnostic criteria for HLH
- prolonged fever > 7 days
- splenomegaly
- bi-cytopenia
- hypertriglyceridemia/hypofibrinogenemia
- hemophagocytosis in BM, spleen, lymph nodes, liver - least specific/sensitive
- low/absent NK cell activity
- elevated soluble IL-2 receptor
- ferritin > 500
what is a potentially lethal complication of Juvenile idiopathic arthritis
- macrophage activating syndrome
if the pathologic inflammation arises from impaired cytotoxic lymphocytes then what condition is it?
- HLH
if the pathogenic inflammation arises from autoimmunity, then what condition could it be?
what do we use to treat it?
- macrophage activation syndrome
- anti-IL-1 (Anakinra)
HLH is reported with what malignancies?
- lymphomas/leukemias of T/NK cell lineage
what viruses can cause HLH
which is the most frequent
- EBV - most frequent
- HIV
- CMV
drug causes of HLH
mechanisms
- Blinatumomab - T cell engager against CD3/CD19
- CAR-T cells - patient’s own T cells to fight against tumor antigen
- cause cytokine release syndrome
treatment of cytokine release syndrome
- tocilizumab IL-6 receptor inhibitor
HLH associated with ____ has a higher mortality than HLH associated with infections or autoimmune disease
- lymphoma
important diagnostic lab test for HLH
- ferritin
treatment of HLH in children
- HLH-94 protocol
- dexamethasone
- etoposide
treatment of HLH in adults
- high dose steroids
- IVIg
what drug is a new treatment of HLH
mechanisms
- Emapalumab
- monoclonal antibody to IFN-gamma
