Bokhari Syed Faqeer Hussain, Bakht Danyal, Hasan Abdul Haseeb, Abid Muhammad Ali, Amir Maaz, Ali Khawar, Javed Muhammad Arsham, Khilji Faria, Iqbal Asma, Dost Wahidullah
Department of Medicine and Surgery, King Edward Medical University, Lahore 54000, Punjab, Pakistan.
Department of Medicine and Surgery, Quaid-e-Azam Medical College, Bahawalpur, Bahawalpur 54000, Punjab, Pakistan.
World J Clin Oncol. 2025 May 24;16(5):104471. doi: 10.5306/wjco.v16.i5.104471.
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.
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.
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.
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.
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.
原发性心脏淋巴瘤（PCL）是心脏肿瘤中罕见的一种，常因症状不具特异性而诊断较晚。它主要影响免疫功能低下的个体，主要累及心包和右心。晚期诊断易与常见心脏疾病相混淆，导致预后不良。
系统评价利妥昔单抗单独或在各种化疗方案中治疗PCL的疗效。次要目标包括评估形态学亚型、评估治疗方案以及分析以缓解和不良事件为重点的治疗结果。
按照PRISMA指南，在多个数据库中进行了全面的文献检索，包括PubMed、Hinari、科学网和Scopus。纳入了报道利妥昔单抗用于治疗人类PCL的英文研究。研究选择包括初步筛选标题和摘要，随后进行全文审查和数据提取。
本系统评价纳入了33篇涉及36例患者的病例报告。弥漫性大B细胞淋巴瘤是观察到的主要形态学亚型。利妥昔单抗、环磷酰胺、阿霉素、长春新碱和泼尼松龙方案是最常用的治疗策略，表明在PCL治疗中得到广泛认可且有效。包括手术干预在内的联合治疗在实现完全缓解方面显示出前景，而一些研究报告称尽管采取了积极的治疗方法仍有死亡病例。
利妥昔单抗，特别是与化疗方案联合使用时，代表了PCL治疗的重大进展，为改善患者预后带来了希望。然而，诸如治疗反应多变和不良事件等挑战凸显了PCL治疗的复杂性。有必要进一步研究以完善治疗策略并加强对这种罕见心脏恶性肿瘤的诊断方法。
