Primary cardiac lymphoblastic B‑cell lymphoma : should we treat more intensively?

Abstract

Primary cardiac lymphoma (PCL) is a rare neoplasm, the majority of cases of which are non‑Hodgkin’s, diffuse large B‑cell (DLBCL). We report the first case of an adult with PCL B‑cell lymphoblastic lymphoma whose disease evolution was grim. A 52‑year‑old male reported dyspnea and facial swelling lasting for 4 months and upon a physical examination he presented bradycardia, jugular venous engorgement, and hypophonesis of cardiac sounds. An electrocardiography (Echo) revealed a right atrial mass and nodules at the pericardium. The patient was treated with R‑Hyper‑CVAD (rituximab plus cyclophosphamide, vincristine, doxorubicin, and dexamethasone) and presented very short remission. At this time, we used R‑ICE (rituximab plus ifosfamide, carboplatin, and etoposide) chemotherapy and the patient underwent complete remission after two courses and received autologous bone marrow transplantation (auto‑BMT). After 75 days of follow‑up, the patient reported dyspnea and a new Echo showed a recurrence of the disease. The patient died due to cardiac failure. PCL is a rare disease with an unfavorable prognosis and a prompt diagnosis and treatment are fundamental to survival. We believe that more intensive therapies, such as auto‑BMT, should be considered as a first treatment option.