Oncology: Langerhans cell histiocytosis: 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.

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Pediatrics · Oncology: Langerhans cell histiocytosis

Sample item

A 2-year-old has seborrhoeic-like scalp rash, lytic skull lesions and diabetes insipidus. Which finding on electron microscopy or immunostaining confirms the diagnosis?

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

The answer and the reasoning

C. Birbeck granules with CD1a positivity

Langerhans cell histiocytosis is a clonal proliferation of dendritic cells that stain for CD1a and langerin and have Birbeck (tennis-racket) granules. Hand-Schuller-Christian disease features the triad of lytic bone lesions, exophthalmos and diabetes insipidus. Treatment ranges from curettage to vinblastine and steroids.

Option by option

  • A. Reed-Sternberg cells

    Reed-Sternberg cells are found in Hodgkin lymphoma.

  • B. Starry-sky macrophages

    Starry-sky pattern is seen in Burkitt lymphoma.

  • C. Birbeck granules with CD1a positivity (correct)

    Correct. CD1a positive cells with Birbeck granules.

  • D. Auer rods

    Auer rods are seen in acute myeloid 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.

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Questions about this Pediatrics question

What is the answer to this Pediatrics question?

The best answer is C, Birbeck granules with CD1a positivity. Langerhans cell histiocytosis is a clonal proliferation of dendritic cells that stain for CD1a and langerin and have Birbeck (tennis-racket) granules.

Why is "Reed-Sternberg cells" not the answer?

Reed-Sternberg cells are found in Hodgkin lymphoma.

Why is "Starry-sky macrophages" not the answer?

Starry-sky pattern is seen in Burkitt lymphoma.

Why is "Auer rods" not the answer?

Auer rods are seen in acute myeloid leukaemia.

What concept does this question test?

It tests Oncology: Langerhans cell histiocytosis in Pediatrics. Hand-Schuller-Christian disease features the triad of lytic bone lesions, exophthalmos and diabetes insipidus. Treatment ranges from curettage to vinblastine and steroids.

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 Oncology: Langerhans cell histiocytosis?

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.