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Primary cardiac lymphoblastic T cell lymphoma
  1. dierk.werner{at}

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A 52 year old man was admitted to our hospital because of ventricular tachycardias. After electrical cardioversion, the patient reported a weight loss of 7 kg and nocturnal sweating during the previous weeks. Laboratory investigations showed moderate anaemia and a slightly raised white blood count with normal differential count. Echocardiography revealed thickened and hypokinetic posterior and lateral walls of the left ventricle. Ultrafast computed tomography (CT) confirmed the thickened walls (top right), which rapidly increased from 16 mm to 30 mm end diastolic diameter over the next two weeks. Coronary angiography detected de novo formation of tumour vessels in the apical wall of the left ventricle. Endomyocardial biopsy from the thickened left ventricular wall was performed and revealed lymphoma. Immunophenotyping demonstrated T cell lymphoblastic lymphoma. Cranial and abdominal CT, thoracic nuclear magnetic resonance tomography, bronchoscopy, as well as bone marrow and spinal fluid specimens revealed no other manifestation of the lymphoma. Polychemotherapy was started with cyclophosphamide, vincristine, daunorubicine, prednisone and, later, L-asparaginase, and was well tolerated. After six days, thickness of the infiltrated wall had decreased to 15 mm and left ventricular function had improved.

 Nineteen days later the patient died suddenly because of interminable ventricular fibrillation. Necropsy findings showed extensive lymphomatous infiltration and necrosis of the posterior and lateral wall of the left ventricle (bottom right) which may have been the focus for the renewed ventricular fibrillation.

Hence, in this case ventricular arrhythmias caused by extensive necrosis limited survival in spite of the therapeutic effect of polychemotherapy.