## **Core Concept**
The question describes a clinical scenario suggestive of a hereditary hemolytic anemia, characterized by chronic hemolysis, abdominal pain (possibly due to vaso-occlusive crises), and abnormal red blood cell (RBC) morphology. This combination of symptoms is highly suggestive of **Sickle Cell Disease (SCD)**, a genetic disorder affecting hemoglobin.
## **Why the Correct Answer is Right**
Sickle Cell Disease is caused by a mutation in the HBB gene that codes for the beta-globin subunit of hemoglobin, leading to the production of **sickle hemoglobin (HbS)**. Under low oxygen conditions, HbS polymerizes, causing RBCs to assume a sickle shape. These sickled RBCs are prone to premature destruction (hemolysis) and can cause vaso-occlusive crises, leading to tissue ischemia and pain. The condition is more prevalent in individuals of African, Caribbean, and Middle Eastern descent. The presence of sickle-shaped RBCs on a peripheral smear is diagnostic.
## **Why Each Wrong Option is Incorrect**
- **Option A:** While other hemoglobinopathies can cause hemolytic anemia, they do not typically present with the combination of vaso-occlusive crises and sickle-shaped RBCs.
- **Option B:** This option does not provide a specific disorder related to the symptoms described.
- **Option C:** Similar to Option A, without specifying a disorder, it's hard to directly refute, but given the context, it's less likely to be correct than Option D.
- **Option D:** Not applicable as it's the correct answer.
## **Clinical Pearl / High-Yield Fact**
A key clinical pearl is that patients with Sickle Cell Disease are at increased risk of infections, particularly those caused by encapsulated organisms like Streptococcus pneumoniae, due to functional asplenia resulting from repeated splenic infarctions. Therefore, vaccination against pneumococcus is crucial in these patients.
## **Correct Answer:** D. Sickle Cell Disease
Free Medical MCQs Β· NEET PG Β· USMLE Β· AIIMS
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