Haematology: sickle cell anaemia treatment: 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 treatment

Sample item

A 9-year-old child with sickle cell anaemia has frequent painful crises and two episodes of acute chest syndrome in a year. Which drug reduces the frequency of these complications, and how?

AErythropoietin, by increasing red cell productionBDeferasirox, by removing iron deposited in tissuesCWarfarin, by inhibiting vitamin K dependent clotting factorsDHydroxyurea, by raising fetal haemoglobin and reducing HbS polymerisation

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

The answer and the reasoning

D. Hydroxyurea, by raising fetal haemoglobin and reducing HbS polymerisation

Sickling is caused by polymerisation of deoxygenated HbS, which results from a glutamic acid to valine substitution at position 6 of the beta chain. Fetal haemoglobin interferes with polymerisation. Hydroxyurea increases HbF and so reduces pain crises, acute chest syndrome and the need for transfusion.

Option by option

A. Erythropoietin, by increasing red cell production
Erythropoietin does not prevent sickling, and it could increase red cell mass and blood viscosity.
B. Deferasirox, by removing iron deposited in tissues
Deferasirox treats transfusional iron overload, but does not prevent sickling.
C. Warfarin, by inhibiting vitamin K dependent clotting factors
Warfarin is not indicated for crisis prevention, as the basis of crises is vaso-occlusion by sickled cells.
D. Hydroxyurea, by raising fetal haemoglobin and reducing HbS polymerisation (correct)
Correct. Higher HbF levels dilute HbS and interfere with its polymerisation.

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.

Questions about this Pathology question

What is the answer to this Pathology question?

The best answer is D, Hydroxyurea, by raising fetal haemoglobin and reducing HbS polymerisation. Sickling is caused by polymerisation of deoxygenated HbS, which results from a glutamic acid to valine substitution at position 6 of the beta chain.

Why is "Erythropoietin, by increasing red cell production" not the answer?

Erythropoietin does not prevent sickling, and it could increase red cell mass and blood viscosity.

Why is "Deferasirox, by removing iron deposited in tissues" not the answer?

Deferasirox treats transfusional iron overload, but does not prevent sickling.

Why is "Warfarin, by inhibiting vitamin K dependent clotting factors" not the answer?

Warfarin is not indicated for crisis prevention, as the basis of crises is vaso-occlusion by sickled cells.

What concept does this question test?

It tests Haematology: sickle cell anaemia treatment in Pathology. Fetal haemoglobin interferes with polymerisation. Hydroxyurea increases HbF and so reduces pain crises, acute chest syndrome and the need for transfusion.

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 treatment?

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.