Haematology: sickle cell anaemia and hyposplenism: 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 · Haematology: sickle cell anaemia and hyposplenism

Sample item

A 7-year-old with sickle cell anaemia has had two episodes of pneumococcal septicaemia. A blood film shows Howell-Jolly bodies and target cells. What is the mechanism behind his susceptibility?

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

The answer and the reasoning

B. Functional asplenia from repeated splenic infarction

Repeated microvascular occlusion infarcts the spleen, which is fibrosed and shrunken by late childhood (autosplenectomy). The loss of splenic clearance of encapsulated bacteria such as Streptococcus pneumoniae and Haemophilus influenzae causes overwhelming sepsis, and Howell-Jolly bodies show that the spleen is no longer removing nuclear remnants. Pneumococcal vaccination and penicillin prophylaxis are therefore given.

Option by option

  • A. Deficiency of the classical complement component C1q

    C1q deficiency predisposes to lupus-like disease and not specifically to this pattern with Howell-Jolly bodies.

  • B. Functional asplenia from repeated splenic infarction (correct)

    Infarction destroys splenic function, leaving encapsulated bacteria uncleared.

  • C. Splenic sequestration with hypersplenism

    Sequestration crisis occurs in young children with an enlarged spleen and causes acute anaemia, not recurrent sepsis in an infarcted spleen.

  • D. Defective neutrophil oxidative burst

    A defective oxidative burst is the defect in chronic granulomatous disease, with catalase-positive infections.

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, Functional asplenia from repeated splenic infarction. Repeated microvascular occlusion infarcts the spleen, which is fibrosed and shrunken by late childhood (autosplenectomy).

Why is "Deficiency of the classical complement component C1q" not the answer?

C1q deficiency predisposes to lupus-like disease and not specifically to this pattern with Howell-Jolly bodies.

Why is "Splenic sequestration with hypersplenism" not the answer?

Sequestration crisis occurs in young children with an enlarged spleen and causes acute anaemia, not recurrent sepsis in an infarcted spleen.

Why is "Defective neutrophil oxidative burst" not the answer?

A defective oxidative burst is the defect in chronic granulomatous disease, with catalase-positive infections.

What concept does this question test?

It tests Haematology: sickle cell anaemia and hyposplenism in Pathology. The loss of splenic clearance of encapsulated bacteria such as Streptococcus pneumoniae and Haemophilus influenzae causes overwhelming sepsis, and Howell-Jolly bodies show that the spleen is no longer removing nuclear remnants. Pneumococcal vaccination and penicillin prophylaxis are therefore given.

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: sickle cell anaemia and hyposplenism?

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.