Haematology: hereditary spherocytosis: 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.

Try it

Pathology · Haematology: hereditary spherocytosis

Sample item

A 12-year-old boy has intermittent jaundice, splenomegaly and pigment gallstones. His father had a splenectomy in childhood. The smear shows spherocytes, MCHC is raised and reticulocytes are increased. Which finding best distinguishes his condition from autoimmune haemolytic anaemia?

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

The answer and the reasoning

B. A negative direct antiglobulin (Coombs) test

Hereditary spherocytosis is caused by defects of red cell membrane skeletal proteins such as ankyrin, spectrin and band 3. The red cells lose membrane, become spherical and are removed by the spleen. Spherocytes with increased MCHC also occur in warm autoimmune haemolytic anaemia, so the negative direct antiglobulin test is what separates the two.

Option by option

  • A. Increased reticulocyte count

    Reticulocytosis occurs in any haemolytic anaemia with a healthy marrow.

  • B. A negative direct antiglobulin (Coombs) test (correct)

    Correct. Coombs negativity points to an intrinsic membrane defect and not antibody coating.

  • C. Raised indirect bilirubin and low haptoglobin

    Raised indirect bilirubin and low haptoglobin are common to all haemolysis.

  • D. A raised mean corpuscular haemoglobin concentration

    A raised MCHC occurs in both conditions because spherocytes are dense.

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 Pathology questions

Questions about this Pathology question

What is the answer to this Pathology question?

The best answer is B, A negative direct antiglobulin (Coombs) test. Hereditary spherocytosis is caused by defects of red cell membrane skeletal proteins such as ankyrin, spectrin and band 3.

Why is "Increased reticulocyte count" not the answer?

Reticulocytosis occurs in any haemolytic anaemia with a healthy marrow.

Why is "Raised indirect bilirubin and low haptoglobin" not the answer?

Raised indirect bilirubin and low haptoglobin are common to all haemolysis.

Why is "A raised mean corpuscular haemoglobin concentration" not the answer?

A raised MCHC occurs in both conditions because spherocytes are dense.

What concept does this question test?

It tests Haematology: hereditary spherocytosis in Pathology. The red cells lose membrane, become spherical and are removed by the spleen. Spherocytes with increased MCHC also occur in warm autoimmune haemolytic anaemia, so the negative direct antiglobulin test is what separates the two.

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 Haematology: hereditary spherocytosis?

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.