Knowledge Check • The Blood Project

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

Fill in the blue field

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Splenomegaly

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

Hematuria

Fever

Elevated ferritin level

Epstein Barr viral load

Hepatomegaly

Cytopenias

Low or no NK cell activity

Elevated sCD25 (sIL-2R)

Hemophagocytosis

Mental status changes

Splenomegaly

Hypofibrinogenemia

Transaminitis

Hypertriglyceridemia

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

What are the thresholds for cytopenias in the HLH-2004 score?

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Hb < 9 g/dL
Platelets < 100 x 10⁹/L
Neutrophils < 1 x 10⁹/L

Of the eight HLH-2004 criteria, which is least widely available?

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NK cell activity – not available in most centers.

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.