Systemic pathology: Rosai-Dorfman disease: practice question

A Pathology 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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Pathology · Systemic pathology: Rosai-Dorfman disease

Sample item

A 12-year-old boy has massive painless bilateral cervical lymphadenopathy with fever and polyclonal hypergammaglobulinaemia. A node biopsy shows dilated sinuses filled with large histiocytes that contain intact lymphocytes. The histiocytes are S-100 positive and CD1a negative. What is the diagnosis?

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

The answer and the reasoning

B. Rosai-Dorfman disease

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) shows sinus expansion by large S-100 positive histiocytes exhibiting emperipolesis, with CD1a negative staining. Langerhans cell histiocytosis is CD1a and langerin positive with grooved nuclei and Birbeck granules.

Option by option

  • A. Langerhans cell histiocytosis

    Langerhans cell histiocytosis is CD1a positive with coffee-bean nuclei.

  • B. Rosai-Dorfman disease (correct)

    Correct. Emperipolesis with S-100 positive, CD1a negative histiocytes identifies Rosai-Dorfman disease.

  • C. Castleman disease

    Castleman disease shows abnormal follicles and vascular proliferation, not sinus histiocytes engulfing lymphocytes.

  • D. Nodular sclerosis Hodgkin lymphoma

    Nodular sclerosis Hodgkin lymphoma shows lacunar cells with fibrous bands and CD15 and CD30 positive Reed-Sternberg cells.

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 Pathology question

What is the answer to this Pathology question?

The best answer is B, Rosai-Dorfman disease. Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) shows sinus expansion by large S-100 positive histiocytes exhibiting emperipolesis, with CD1a negative staining.

Why is "Langerhans cell histiocytosis" not the answer?

Langerhans cell histiocytosis is CD1a positive with coffee-bean nuclei.

Why is "Castleman disease" not the answer?

Castleman disease shows abnormal follicles and vascular proliferation, not sinus histiocytes engulfing lymphocytes.

Why is "Nodular sclerosis Hodgkin lymphoma" not the answer?

Nodular sclerosis Hodgkin lymphoma shows lacunar cells with fibrous bands and CD15 and CD30 positive Reed-Sternberg cells.

What concept does this question test?

It tests Systemic pathology: Rosai-Dorfman disease in Pathology. Langerhans cell histiocytosis is CD1a and langerin positive with grooved nuclei and Birbeck granules.

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 pathology: Rosai-Dorfman disease?

Learn each disease as a bundle: cause, key histology, markers, and the clinical hallmark. Then use the flashcards and the other Pathology 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.