P281 CARDIAC RICHTER‘S SYNDROME: A RARE CASE OF CHRONIC LYMPHOCYTIC LEUKAEMIA TRANSFORMATION

Abstract A 71–year–old woman with chronic lymphocytic leukaemia (CLL) presented to our Cardiology clinic complaining of recent–onset chest pain and dysphonia. Physical examination ECG were normal. blood test revealed high levels high–sensitivity troponin T (373 ng/L) C–reactive protein (7 mg/dL); counts differential Transthoracic echocardiography showed a hyperechogenic appearance the interatrial septum, interventricular septum right ventricular free wall, as well mild circumferential pericardial effusion. Cardiac magnetic resonance imaging multiple intracardiac masses involving both ventricles evidence oedema fibrosis. computed tomography presence cervical, pulmonary, mediastinal masses, one which was biopsied through video–thoracoscopy. At histological examination, mass mainly consisted large pleomorphic peripheral CD10–, CD20+/–, CD79a+, PAX5+, BCL2+, BCL6–, c–MYC+ B cells, proliferative activity (Ki–67/MIB1+ 80%). diagnosis CLL transformation into diffuse B–cell lymphoma (DLBCL), also known Richter’s syndrome (RS), established (Figure). Despite indication for specific haematological chemotherapy, patient rapidly deteriorated died few days after diagnosis. RS is rare evolution small lymphoma, affecting 2–8% such patients (transformation <1%/ year), yet burdened very poor prognosis (median overall survival 8–16 months). Extra–nodal uncommon, cardiac involvement has been reported in only 3 cases literature (in none panel techniques here used). its rarity, worsening symptoms should be suspected heart. The main primary lymphoma. characterization relies on multimodality imaging, but histologic analysis required reach definite diagnosis, essential guide subsequent therapeutic choices.