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Haematology Radiology What is the diagnosis?

What is the diagnosis?

image
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.


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