Board Questions

1. Which of the following is NOT part of the diagnostic criteria for HLH? 

a) Elevated Ferritin
b) Low/absent NK cell function
c) Elevated IL-2 receptor (sCD25) level
d) Elevated IL-1 level

2. Which of the following is not a treatment for HLH? 

a) Hematopoietic cell transplant
b) Dexamethasone
c) Prednisone
d) Etoposide

3.Which of the following organs seems to be more affected in MAS than typically occurs with HLH? 

a) Heart
b) Lungs
c) Liver
d) Kidney

4. Which is thought of as the best marker to follow severity/progression of HLH?

a) Ferritin
b) Fibriongen
c) NK cell function
d) sCD25 (soluble IL2R alpha)

ANSWERS & EXPLANATIONS

1. D- While IL-1 is certainly elevated with HLH, it is nonspecific and not part of the diagnostic criteria for HLH. The rest of the choices are criteria for the diagnosis of HLH.

2.C- while steroids are used for treatment of HLH, dexmethasone is preferred given its ability to cross the brain blood barrier. Hence, C, prednisone, is not typically used to treat HLH. Etoposide and HCT are both used to treat HLH

3. A- Cardiac impairment is more common with MAS than with HLH. Liver dysfunction is common in both HLH and MAS and the lack of liver inflammation would be unusual with HLH

4. D- sCD25 (soluble IL-2Ralpha) is thought to be the best marker to follow disease progression/course for HLH (hence, it is recommended to be obtained weekly during induction therapy)
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