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Case 36

Thalassaemia major, extramedullary haematopoesis

A young adult male with an upper respiratory infection

This young man was referred by his general practitioner with an upper respiratory tract infection, to exclude a secondary pneumonia.

The patient, a young Asian man, suffered with chronic anaemia. His lateral chest film is shown.

Findings

The PA film demonstrates bilateral mediastinal masses. The left and right heart borders are clearly visible, as are the hilar vessels, implying that the masses are posterior. This is confirmed on the lateral film, where large soft tissue masses are seen overlying the thoracic spine.

Additionally the bones appear markedly abnormal. The ribs are expanded anteriorly, and are osteopaenic with a coarse trabecular pattern.

Diagnosis

Thalassaemia major, extramedullary haematopoesis

Discussion

The posterior mediastinal masses are due to extramedullary aemopoesis. The marrow hyperplasia also accounts for the bone changes. Cardiomegaly may also be a feature on the chest film, secondary to the chronic anaemia.

Such individuals are more prone to infections, because of their general poor health and leukopaenia secondary to splenomegaly.

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