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Sickle Cell Disease — RACP Adult Medicine MCQ

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EasyHaematologySickle Cell DiseaseRACP Adult Medicine

A 25-year-old African man presents with acute severe pain crisis (chest, back, limbs), Hb 68 g/L, and reticulocyte count 15%. Blood film shows sickle cells, target cells, Howell-Jolly bodies, and polychromasia. Hb electrophoresis shows HbS 85%, HbF 8%, HbA₂ 3.5%, HbA 0%. What is the most likely blood film diagnosis?

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Correct answer: C — Sickle cell disease

Sickle cells on blood film with vaso-occlusive pain crisis, Howell-Jolly bodies (functional asplenia), and HbS 85% with absent HbA on electrophoresis confirms sickle cell disease (HbSS). Howell-Jolly bodies indicate autosplenectomy. Acute management includes opioid analgesia, IV fluids, oxygen, and transfusion if indicated. Hydroxyurea reduces crisis frequency.

Reference: HSANZ – 2024 – SCD Guidelines; BSH 2022 SCD