Question
What is the diagnosis?
- Beta-thalassemia
- Eisenmenger syndrome
- Metastatic small cell lung carcinoma
- Sarcoidosis
- Scleroderma
Correct answer : Beta-thalassemia
Explanation
There is marked medullary expansion of the bony structures suggestive of compensatory extramedullary hematopoiesis. The pulmonary arteries are enlarged suggestive of pulmonary hypertension. Together these findings are most consistent with beta-thalassemia.
Pulmonary hypertension in these patients is thought to arise from chronic anemia, hemolysis, and an increased tendency for microscopic thrombi to form within the pulmonary vasculature.