Haemophagocytic Lymphohistiocytosis — SCE Infectious Diseases MCQ

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HardImmunocompromised HostHaemophagocytic LymphohistiocytosisSCE Infectious Diseases

A patient has persistent fever, splenomegaly, pancytopenia, ferritin 28,000 micrograms/L and haemophagocytosis after acute EBV infection. What is the most likely syndrome?

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Correct answer: D — EBV-triggered haemophagocytic lymphohistiocytosis

The best answer is “EBV-triggered haemophagocytic lymphohistiocytosis”. The constellation of extreme hyperferritinaemia, cytopenias, organomegaly and haemophagocytosis indicates HLH; urgent specialist treatment addresses both the inflammatory syndrome and its EBV trigger. HLH is a hyperinflammatory syndrome identified from fever, cytopenias, organomegaly, ferritin and immune activation; infection including EBV can trigger life-threatening disease. The UK adult HLH pathway supports urgent specialist confirmation and treatment of secondary HLH, including infection-triggered disease.

Reference: EULAR/ACR points to consider for HLH and macrophage-activation syndrome: https://ard.bmj.com/content/82/10/1271 NHS England commissioned adult HLH service: https://www.uclh.nhs.uk/our-services/find-service/medical-specialties-1/hlh-service