Cardiac Infiltration in Mycosis Fungoides Resulting in Recurrent Tamponade

Clinically apparent cardiac involvement in mycosis fungoides (MF) is a rare phenomenon.

A 61-year-old man presented with dyspnea 11 months after allogeneic hematopoietic stem cell transplantation for MF. Echocardiography revealed a large pericardial effusion causing cardiac tamponade. Emergency pericardiocentesis and effusion analysis confirmed disease relapse isolated to the pericardium in the setting of full donor chimerism. Despite reduction in immunosuppression, relapse with cardiac tamponade later recurred. Combined-modality chemotherapy and radiotherapy was administered. Systemic disease relapse occurred 22 months after isolated pericardial relapse.

This unique case of life-threatening isolated cardiac involvement in relapsed MF exposes the heart as a potential sanctuary site that may sequester lymphoma cells from systemic therapies and the graft-versus-lymphoma effect. Optimal management of this rare complication is unknown.

Pericardial or myocardial infiltration should be considered in patients with MF presenting with cardiac symptoms, conduction disturbance, or shock.