Haematology: thalassaemia major: 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 · Haematology: thalassaemia major

Sample item

A 1-year-old has severe pallor, hepatosplenomegaly and frontal bossing, with Hb 5 g/dl, microcytic hypochromic indices and HbF raised on electrophoresis. What is the mainstay of management?

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

The answer and the reasoning

C. Regular transfusions with iron chelation

Beta thalassaemia major needs regular red cell transfusions to maintain pre-transfusion Hb at about 9 to 10 g/dl, together with chelation (deferasirox or deferiprone) to prevent iron overload. Hematopoietic stem cell transplantation is curative. Oral iron is contraindicated.

Option by option

  • A. Hydroxyurea only

    Hydroxyurea is not standard for transfusion-dependent disease.

  • B. Oral iron

    Oral iron worsens overload.

  • C. Regular transfusions with iron chelation (correct)

    Correct. Transfusion with chelation is standard.

  • D. Splenectomy at diagnosis

    Splenectomy is reserved for hypersplenism.

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, Regular transfusions with iron chelation. Beta thalassaemia major needs regular red cell transfusions to maintain pre-transfusion Hb at about 9 to 10 g/dl, together with chelation (deferasirox or deferiprone) to prevent iron overload.

Why is "Hydroxyurea only" not the answer?

Hydroxyurea is not standard for transfusion-dependent disease.

Why is "Oral iron" not the answer?

Oral iron worsens overload.

Why is "Splenectomy at diagnosis" not the answer?

Splenectomy is reserved for hypersplenism.

What concept does this question test?

It tests Haematology: thalassaemia major in Pediatrics. Hematopoietic stem cell transplantation is curative. Oral iron is contraindicated.

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: thalassaemia major?

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.