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Multi Organ Synthesis > MAS > Flashcards

MAS Flashcards

1
Q

what does HLH stand for?

A

hemophagocytic lymphohistiocytosis

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2
Q

define HLH

A

multi system disorder characterized by dysregulated immune system and hypercytokinemia

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3
Q

what type of HLH shows up in kids?

A

primary…from genetic defects

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4
Q

what type of HLH shows up in adults?

A

secondary…acquired

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5
Q

what do macros do that is odd in HLH?

A

hemophagocytosis

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6
Q

what iron thing can be elevated in HLH?

A

ferritin

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7
Q

what can happen with temp in HLH?

A

fever

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8
Q

what can happen to spleen in HLH?

A

splenomegaly

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9
Q

what can happen to blood counts in HLH?

A

bi cytopenia

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10
Q

what cell may be super low in activity in HLH?

A

NK cells

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11
Q

what cytokine can be elevated in HLH?

A

aIL2R

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12
Q

what two cells are likely hyperactivated that start the physiologic process of HLH?

A

hyperactivation of Cd8 and macros

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13
Q

what does MAS stand for?

A

macrophage activation syndrome

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14
Q

what disease is MAS associated with?

A

JIA

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15
Q

what is main difference in HLH and MAs?

A

MAS is from autoimmunity activation and HLH is from impaired cytotoxic lymphocytes

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16
Q

what is advantage of MAS compared to HLH in terms of therapy?

A

if it arises from specific autoimunity issues then we may be able to target therapt

17
Q

what malignancies are associated with HLH?

A

lymphomas/leukemias of T and NK cell

18
Q

what is most frequent infection associated with HLH?

A

EBV

19
Q

what are two other viral infections that cause HLH?

A

HIV and CMV

20
Q

what is a super important lab in diagnosing HLH?

A

ferritin

21
Q

what three things can be elevated in blood in HLH?

A

ferritin
triglycerides
fibrinogen

22
Q

what is Rx for HLH?

A

high dose steroids…often dexamethasone

23
Q

what can you target in therapy for HLH?

A

any cytokines that are elevated…say IL-1 is up we can use anakinra

24
Q

what is the HLH 94 rx used for?

A

for kids with HLH

25
Q

what drugs are in HLH94 protocol?

A

dexamethasone and etoposide

26
Q

what is target of etoposide?

A

activated T cells

Multi Organ Synthesis (9 decks)

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