MAS Flashcards
what does HLH stand for?
hemophagocytic lymphohistiocytosis
define HLH
multi system disorder characterized by dysregulated immune system and hypercytokinemia
what type of HLH shows up in kids?
primary…from genetic defects
what type of HLH shows up in adults?
secondary…acquired
what do macros do that is odd in HLH?
hemophagocytosis
what iron thing can be elevated in HLH?
ferritin
what can happen with temp in HLH?
fever
what can happen to spleen in HLH?
splenomegaly
what can happen to blood counts in HLH?
bi cytopenia
what cell may be super low in activity in HLH?
NK cells
what cytokine can be elevated in HLH?
aIL2R
what two cells are likely hyperactivated that start the physiologic process of HLH?
hyperactivation of Cd8 and macros
what does MAS stand for?
macrophage activation syndrome
what disease is MAS associated with?
JIA
what is main difference in HLH and MAs?
MAS is from autoimmunity activation and HLH is from impaired cytotoxic lymphocytes
what is advantage of MAS compared to HLH in terms of therapy?
if it arises from specific autoimunity issues then we may be able to target therapt
what malignancies are associated with HLH?
lymphomas/leukemias of T and NK cell
what is most frequent infection associated with HLH?
EBV
what are two other viral infections that cause HLH?
HIV and CMV
what is a super important lab in diagnosing HLH?
ferritin
what three things can be elevated in blood in HLH?
ferritin
triglycerides
fibrinogen
what is Rx for HLH?
high dose steroids…often dexamethasone
what can you target in therapy for HLH?
any cytokines that are elevated…say IL-1 is up we can use anakinra
what is the HLH 94 rx used for?
for kids with HLH
