Haemophagocytic Lymphohistiocytosis – Board-Style Questions

What this deck covers

Haemophagocytic Lymphohistiocytosis in 18 board-style clinical cases with worked explanations. Each case gives you a clinical vignette, four options, the correct answer, and an explanation of why each of the other three is wrong, with the guideline or textbook it follows named on the answer.

Board-style teaching questions for paediatricians and trainees preparing for paediatric board, MRCPCH and licensing examinations. Written from published guidelines and standard paediatric textbooks, with the source named on every answer.

The 18 cases, in order

1. Fever that will not settle
2. Beyond the eight
3. A seizure on the ward
4. No engulfed cells
5. The send-away pair
6. Two variants, three signs
7. Before the sequencing
8. A sister lost in infancy
9. Pigment in the shaft
10. His mother’s brother
11. The usual culprit
12. Organisms in the marrow
13. Nodes behind the gut
14. Hold for the genes?
15. Known systemic arthritis
16. Still burning
17. Remission after a virus
18. A healthy new baby

Diagnoses and management options tested

Across the 18 cases you are asked to choose between options such as: Enlargement of the spleen below the costal margin, A bone marrow trephine looking for haemophagocytosis, The LYST gene of Chediak-Higashi syndrome only, Chediak-Higashi syndrome, a LYST gene defect, Familial HLH from biallelic perforin gene variants, Parvovirus B19, by a single serology test for IgM, Systemic lupus erythematosus with renal involvement, Etoposide and dexamethasone on the HLH-94 protocol.

Clinical pearls from this deck

• Fever, spleen and falling counts that antibiotics cannot touch: send a ferritin.
• The liver is almost always involved in HLH, yet it never earns a point on the criteria.
• In HLH, look at the brain and the spinal fluid – but make the clotting safe first.
• Haemophagocytes are a supporting witness, not the whole case.
• The two send-away criteria of HLH: soluble CD25 up, NK activity down.
• In HLH, the gene can make the diagnosis on its own.

Work through the deck once for recognition, then again a week later to test yourself — each question gives the answer and explains why every other option fails.

More from this system: all Haematology case decks · pediatric reference values.