Haematology: myeloproliferative neoplasms: practice question

A General Medicine 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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General Medicine · Haematology: myeloproliferative neoplasms

Sample item

A 55-year-old man has facial plethora, an itchy skin after a hot bath, splenomegaly and a haemoglobin of 19 g/dL with a low serum erythropoietin. Which mutation is most likely, and what is the first-line treatment?

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

The answer and the reasoning

B. JAK2 V617F, with phlebotomy and low-dose aspirin

Polycythaemia vera is driven by a JAK2 mutation (V617F in about 95%) and causes erythrocytosis with low erythropoietin, aquagenic pruritus and thrombotic risk. Phlebotomy to keep haematocrit below 45% and low-dose aspirin are first-line. Hydroxyurea or ruxolitinib are used in high-risk patients.

Option by option

  • A. MPL, with splenectomy

    MPL mutations are rare in these conditions and splenectomy is not first-line.

  • B. JAK2 V617F, with phlebotomy and low-dose aspirin (correct)

    Correct. A JAK2 mutation, low erythropoietin and phlebotomy define the approach.

  • C. CALR, with plasmapheresis

    CALR mutations occur in essential thrombocythaemia and myelofibrosis, and plasmapheresis is not standard.

  • D. BCR-ABL, with imatinib

    BCR-ABL defines chronic 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.

More General Medicine questions

Questions about this General Medicine question

What is the answer to this General Medicine question?

The best answer is B, JAK2 V617F, with phlebotomy and low-dose aspirin. Polycythaemia vera is driven by a JAK2 mutation (V617F in about 95%) and causes erythrocytosis with low erythropoietin, aquagenic pruritus and thrombotic risk.

Why is "MPL, with splenectomy" not the answer?

MPL mutations are rare in these conditions and splenectomy is not first-line.

Why is "CALR, with plasmapheresis" not the answer?

CALR mutations occur in essential thrombocythaemia and myelofibrosis, and plasmapheresis is not standard.

Why is "BCR-ABL, with imatinib" not the answer?

BCR-ABL defines chronic myeloid leukaemia.

What concept does this question test?

It tests Haematology: myeloproliferative neoplasms in General Medicine. Phlebotomy to keep haematocrit below 45% and low-dose aspirin are first-line. Hydroxyurea or ruxolitinib are used in high-risk patients.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. 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 Haematology: myeloproliferative neoplasms?

For each disease know the classic presentation, the best test, and the first-line treatment. Then use the flashcards and the other General Medicine 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.