Biatrial Double-Hit Lymphoma Presenting with Superior Vena Cava Syndrome

Cardiac involvement is very rare in lymphomas and is typically characterized by a poor prognosis, largely due to delay in diagnosis and cardiac complications or venous/arterial thromboembolism. This unusual presentation and other extranodal localizations are more likely to be found in aggressive lymphomas rather than indolent diseases. In this report we present the case of a patient with heart involvement by a biatrial double mass conditioning superior vena cava syndrome. Histological findings consistent with double hit lymphoma could explain the very aggressive behaviour and the extranodal tropism of this lymphoma. We are treating this patient with an intensified chemotherapy according to EPOCH regimen under carefully clinical and cardiological monitoring.