Rituximab-based regimens for primary cardiac lymphoma: A systematic review of outcomes, challenges and future directions.

Abstract

Background: Primary cardiac lymphoma (PCL) is a rare subset of cardiac tumors, often diagnosed late due to nonspecific symptoms. It predominantly affects immunocompromised individuals, primarily in the pericardium and right heart. Late diagnosis mimics common cardiac ailments, leading to poor prognosis.

Aim: To systematically review the efficacy of rituximab in treating PCL either alone or in various chemotherapeutic regimens. Secondary objectives include evaluating morphological subtypes, assessing treatment regimens, and analyzing outcomes focusing on remission and adverse events.

Methods: Following PRISMA guidelines, a comprehensive literature search was conducted across multiple databases, including PubMed, Hinari, Web of Science, and Scopus. English-language studies reporting the use of rituximab in treating PCL in humans were included. Study selection involved initial screening of titles and abstracts followed by full-text examination and data extraction.

Results: Thirty-three case reports involving 36 patients were included in this systematic review. Diffuse large B-cell lymphoma was the predominant morphological subtype observed. The rituximab, cyclophosphamide, doxorubicin, oncovin, and prednisolone regimen emerged as the most commonly employed treatment strategy, indicating widespread acceptance and efficacy in PCL management. Combination therapies, including surgical intervention, showed promise in achieving complete remission, while some studies reported mortality despite aggressive treatment approaches.

Conclusion: Rituximab, particularly in combination with chemotherapy regimens, represents a significant advancement in PCL management, offering hope for improved patient outcomes. However, challenges such as variable treatment responses and adverse events underscore the complexity of managing PCL. Further research is warranted to refine therapeutic strategies and enhance diagnostic approaches for this rare cardiac malignancy.