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Beta-Thalassemia with Extramedullary Hematopoiesis and Diffuse Marrow Expansion

Beta-Thalassemia with Extramedullary Hematopoiesis and Diffuse Marrow Expansion▶ Watch on YouTube — age-restricted, cannot embed here

Imaging findings

Coronal CT and MR images demonstrate striking, diffuse expansion of the medullary spaces of all visualized ribs. Multiple soft-tissue density, iso-intense to muscle paraspinal masses are present along the posterior mediastinum in the paravertebral regions. These masses exhibit heterogeneous enhancement on post-contrast series. Additionally, MR imaging demonstrates that the liver is extremely dark on T2-weighted and gradient echo sequences.

Key takeaways

Diffuse marrow expansion and extramedullary hematopoiesis are classic compensatory responses to chronic severe anemia in patients with beta-thalassemia. Extramedullary hematopoiesis classically manifests as soft-tissue paraspinal masses in the posterior mediastinum. Due to repeated blood transfusions, these patients often develop secondary hemochromatosis, resulting in marked iron deposition and T2-shortening (darkening) of the liver on MRI.

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