Evaluation

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Quiz answers

1. Which clinical and laboratory features should raise early suspicion for HLH/MAS?

A. Fever, splenomegaly, cytopenia, elevated ferritin
B. Fever, leukocytosis, elevated CRP
C. Rash, normal ferritin, thrombocytosis
D. Hepatomegaly, low ESR, elevated platelets

Correct answer: A. Fever, splenomegaly, cytopenia, elevated ferritin. These are hallmark features of HLH/MAS: the combination of systemic symptoms and hyperinflammatory markers is key.

2. Which of the following best describe the basis of the MAS-2016 classification criteria for MAS complicating sJIA?

A. Adapted from the HLH-2004 criteria and validated in adults with malignancy-associated HLH
B. Requires bone marrow biopsy confirmation of hemophagocytosis
C. Developed using expert consensus and statistical comparison of MAS vs sJIA flare presentations
D. Exclude ferritin as a diagnostic marker due to its lack of specificity

Correct answer: C. Developed using expert consensus and statistical comparison of MAS vs sJIA flare presentations. The MAS-2016 criteria, designed specifically for systemic juvenile idiopathic arthritis (sJIA), were informed by statistical analysis of clinical and laboratory features and shaped through expert consensus among pediatric rheumatologists and hematologists.

3. What is the significance of elevated IL-18 in HLH/MAS?

A. It confirms Epstein–Barr virus infection as the trigger
B. It suggests macrophage-driven hyperinflammation
C. It is a marker of neutrophil activation
D. It is a reliable marker for familial HLH

Correct answer: B. It suggests macrophage-driven hyperinflammation. IL-18 is produced by activated macrophages and is associated with macrophage-driven hyperinflammation, which can correlate with disease severity.

4. Which biomarker combination is most useful for distinguishing MAS from sJIA flare?

A. CRP and ESR
B. CXCL9 and IL-18
C. Platelets and LDH
D. IL-6 and TNF-α

Correct answer: B. CXCL9 and IL-18. These biomarkers help distinguish MAS from sJIA flares through their association with interferon gamma and macrophage activation.

5. Which of the following is a key principle in managing HLH/MAS?

A. Delay treatment until diagnosis is confirmed
B. Treat only after genetic testing
C. Initiate early immunomodulation in rapidly worsening cases
D. Use antibiotics alone until hyperinflammation resolves

Correct answer: C. Initiate early immunomodulation in rapidly worsening cases. HLH/MAS can progress quickly, and early immunomodulation is critical even before full diagnostic confirmation.

6. Which three agents are considered pillars of early immunomodulation in HLH/MAS?

A. Methotrexate, IVIg, tocilizumab
B. Glucocorticoids, anakinra, IVIg
C. Cyclophosphamide, anakinra, rituximab
D. Prednisone, azathioprine, IVIgs

Correct answer: B. Glucocorticoids, anakinra, IVIg. These are the three main agents used for early immunomodulation. Glucocorticoids are standard; anakinra and IVIg are often used off-label.