Knowledge Check

The HLH-2004 score was developed primarily for:

a
Children
b
Adults

True of false: no single clinical or laboratory parameter has sensitivity and specificity to definitively detect HLH.

a
True
Hence the use of diagnostic scores like HLH-2004!
b
False

Sort the clinical parameter (on top) into HLH-2004 and non-HLH buckets (at bottom):

Low or no NK cell activity
Splenomegaly
Epstein Barr viral load
Hypofibrinogenemia
Elevated ferritin level
Hematuria
Mental status changes
Hypertriglyceridemia
Transaminitis
Hemophagocytosis
Fever
Elevated sCD25 (sIL-2R)
Hepatomegaly
Cytopenias
HLH-2004 diagnostic criteria
Not part of HLH-2004 diagnostic criteria

What is the threshold level for serum ferritin (ng/ml or mg/L) in the HLH-2004 score?

a
> 100
b
> 200
c
> 500
Hyperferritinemia detected in > 90% of adults. values typically range from > 7,000 mcg/L to 10,000 mcg/L; may be > 100,000 mcg/L on occasion. No specific ferritin level determined to be specific for HLH, but negative predictive value of a normal value thought to be high.
d
> 1,000
e
> 10,000

True of false: the HLH-2004 criteria include elevated serum AST and/or ALT levels.

a
True
b
False
These are included in the HScore.