Haematology: hereditary spherocytosis: 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: hereditary spherocytosis

Sample item

A child has chronic haemolysis, splenomegaly, spherocytes on smear and increased osmotic fragility. A defect in which protein is commonly involved?

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

The answer and the reasoning

A. Spectrin or ankyrin of the red cell membrane

Hereditary spherocytosis is an autosomal dominant defect of the red cell membrane skeleton, usually ankyrin, spectrin or band 3, and the spherocytes are removed in the spleen. Splenectomy is considered in severe cases after 5 to 6 years of age. MCHC is increased and the Coombs test is negative.

Option by option

  • A. Spectrin or ankyrin of the red cell membrane (correct)

    Correct. Membrane skeletal protein defects cause spherocytosis.

  • B. G6PD

    G6PD deficiency causes episodic haemolysis with Heinz bodies.

  • C. Pyruvate kinase

    Pyruvate kinase deficiency gives non-spherocytic haemolysis.

  • D. Haemoglobin beta chain

    A beta globin defect causes haemoglobinopathies.

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 A, Spectrin or ankyrin of the red cell membrane. Hereditary spherocytosis is an autosomal dominant defect of the red cell membrane skeleton, usually ankyrin, spectrin or band 3, and the spherocytes are removed in the spleen.

Why is "G6PD" not the answer?

G6PD deficiency causes episodic haemolysis with Heinz bodies.

Why is "Pyruvate kinase" not the answer?

Pyruvate kinase deficiency gives non-spherocytic haemolysis.

Why is "Haemoglobin beta chain" not the answer?

A beta globin defect causes haemoglobinopathies.

What concept does this question test?

It tests Haematology: hereditary spherocytosis in Pediatrics. Splenectomy is considered in severe cases after 5 to 6 years of age. MCHC is increased and the Coombs test is negative.

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: hereditary spherocytosis?

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.