Primary cardiac lymphoma A comprehensive review

Abstract

Objective: To summarize and analyze the clinical features, treatment and prognosis of patients with primary cardiac lymphoma. Method: We herein review the literature available in PubMed databases for recent five years. Then we analyze and summarize the literature data. Result: Among the 26 patients with primary cardiac lymphoma (PCL), the right atrium was most common site involved, and most tumors were single mass, the main symptoms were dyspnoea（65.4%），chest oppression (38.5%) and edema (19.2%). The elevations of lactate dehydrogenase (LDH) were observed, however the bone marrow aspiration or biopsy? Examinations were negative. Atrial fibrillation and atrioventricular block were also noted in some cases. The common expression of immunophenotyping were CD20, Ki-67, multiple myeloma oncogene 1 (MUM1), B-cell lymphoma/leukemia-6 (Bcl-6), CD10, and might be accompanied by expression of Bcl-2, c-myc, CD3 and CD5, while negative expression of CD30, anaplastic lymphoma kinase (ALK), Epstein-Barr virus encoded small RNA (EBER). The majority of patients in this cohort receiving R±CHOP chemotherapy (cyclophosphamide + pirarubicin + vincristine + prednisone with/without rituximab) were reported to have a poor prognosis with 1-year survival rate at 24.1% (9/26). Conclusions: PCL is very rare, with the variable clinical manifestations potentially leading to a delayed diagnosis.