Systemic paediatrics: haemophagocytic lymphohistiocytosis: practice question

A Pediatrics question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

Try it

Pediatrics · Systemic paediatrics: haemophagocytic lymphohistiocytosis

Sample item

An 18-month-old has fever for 12 days, hepatosplenomegaly and bicytopenia. Ferritin is 8500 ng/mL, triglycerides are 410 mg/dL and fibrinogen is 90 mg/dL. Bone marrow shows histiocytes engulfing blood cells. Which treatment forms the backbone of initial therapy?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

A. Dexamethasone with etoposide

Haemophagocytic lymphohistiocytosis is hyperinflammation from uncontrolled T cell and macrophage activation. Fever, cytopenias, hepatosplenomegaly, high ferritin, high triglycerides and low fibrinogen are diagnostic features. Standard induction uses dexamethasone and etoposide, with transplant for familial disease.

Option by option

  • A. Dexamethasone with etoposide (correct)

    Correct. Dexamethasone with etoposide suppresses the hyperinflammatory state and is the standard induction.

  • B. Intravenous immunoglobulin alone

    Intravenous immunoglobulin alone does not control established haemophagocytic disease.

  • C. Empirical antitubercular therapy

    Antitubercular therapy treats infection, which can trigger the syndrome but is not its main treatment.

  • D. Vincristine, prednisolone and asparaginase

    Vincristine, prednisolone and asparaginase is an induction for acute lymphoblastic leukaemia.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

More Pediatrics questions

Questions about this Pediatrics question

What is the answer to this Pediatrics question?

The best answer is A, Dexamethasone with etoposide. Haemophagocytic lymphohistiocytosis is hyperinflammation from uncontrolled T cell and macrophage activation.

Why is "Intravenous immunoglobulin alone" not the answer?

Intravenous immunoglobulin alone does not control established haemophagocytic disease.

Why is "Empirical antitubercular therapy" not the answer?

Antitubercular therapy treats infection, which can trigger the syndrome but is not its main treatment.

Why is "Vincristine, prednisolone and asparaginase" not the answer?

Vincristine, prednisolone and asparaginase is an induction for acute lymphoblastic leukaemia.

What concept does this question test?

It tests Systemic paediatrics: haemophagocytic lymphohistiocytosis in Pediatrics. Fever, cytopenias, hepatosplenomegaly, high ferritin, high triglycerides and low fibrinogen are diagnostic features. Standard induction uses dexamethasone and etoposide, with transplant for familial disease.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Systemic paediatrics: haemophagocytic lymphohistiocytosis?

Memorise milestones and the vaccine schedule as tables and revise them often. Then use the flashcards and the other Pediatrics questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.