FRCPath Haem Part 1 MCQs-Gen Haem 498

A 22-year-old man is diagnosed with haemophagocytic lymphohistiocytosis (HLH). Despite initial treatment with corticosteroids, he develops persistent fever, worsening hyperferritinaemia and progressive organ dysfunction. Anakinra, a recombinant interleukin-1 (IL-1) receptor antagonist, is commenced as second-line treatment.

After 7 days of treatment, his ferritin concentration has decreased from 20,000 µg/L to 18,000 µg/L. His organ support requirements remain unchanged, and serial HScore measurements have not demonstrated a reduction. Corticosteroid treatment has not yet been reduced.

Which of the following statements best describes the response criteria for anakinra treatment in HLH?

A. A reduction in ferritin of at least 50% within 7 days is required to demonstrate response.

B. A reduction in ferritin of at least 10% within 7 days is one accepted response criterion, and improvement in any one of the specified criteria may demonstrate response.

C. All four response criteria (reduction in H score, steroid, organ dysfunction and ferritin) must be fulfilled before treatment can be considered effective.

D. A reduction in corticosteroid dose of at least 50% within 14 days is mandatory, irrespective of other clinical or biochemical changes.

E. A reduction in HScore is the only accepted measure of response to anakinra.