A presentation, treatment, and survival analysis of primary cardiac lymphoma cases reported from 2009 to 2019
Haoguang Chen1, Shenxian Qian2, Pengfei Shi1
1Institution and Department, Department of Hematology, Hangzhou First People's Hospital Affiliated to Nanjing Medical University, Graduate School of Nanjing Medical University, Shangcheng District, No. 261, Huansha Road, Hangzhou, Zhejiang, China.
Insights
Primary cardiac lymphoma (PCL) is rare but survival has improved. Arrhythmia impacts progression-free survival, and surgery offers no long-term benefit, indicating a need for CNS prophylaxis.
Area of Science:
- Cardiology
- Oncology
- Hematology
Background:
- Primary cardiac lymphoma (PCL) is a rare malignancy with a historically poor prognosis.
- Previous systematic reviews established a framework for understanding PCL, but recent advances necessitate updated analysis.
Purpose of the Study:
- To systematically analyze epidemiological characteristics, clinical presentations, treatments, and outcomes of PCL.
- To provide updated insights into PCL prognostication in light of recent research.
Main Methods:
- Systematic review of cardiac lymphoma case reports published between January 2009 and January 2019.
- Analysis of patient statistics including demographics, clinical manifestations, treatments, and survival data.
- Statistical analysis of progression-free survival and the impact of specific factors like arrhythmia and surgery.
Main Results:
- 158 cases of heart lymphoma were identified, with 101 classified as PCL.
- Diffuse large B-cell lymphoma was the most common type; males predominated.
- Arrhythmia was associated with shorter progression-free survival (HR 0.334, P=0.042), while surgery did not improve long-term outcomes.
- Overall survival for PCL patients has improved compared to historical data (1949-2009).
Conclusions:
- Central nervous system prophylaxis is recommended for PCL patients.
- Current findings suggest a need for revised prognostication strategies for PCL.
- Improved survival rates indicate progress in PCL management.
Abstract:
The heart is a rare primary site of lymphoma, and cardiac involvement is thought to bring a poorer prognosis. A framework of known clinical presentations, diagnostic features, disease complications, treatments, and outcomes to improve prognostication was constructed by a systematic review in 2011. However, some aspects must be discussed further in light of recent advances in lymphoma research. We collected cardiac lymphoma case reports published from January 2009 to January 2019, collected statistics from each patient, and performed a systematic analysis. The epidemiological characteristics, clinical manifestations, treatments, responses, and survival of primary cardiac lymphoma (PCL) patients are described. We obtained 158 cases of heart lymphoma, of which 101 were defined as PCL. There were more male than female cases. Most cases were diffuse large B-cell lymphoma. Six cases of PCL in cardiac myxomas were described. Patients with arrhythmia had shorter progression-free survival compared those without (HR 0.334, 95% CI 0.112-0.999, log-rank P = 0.042). Surgery did not improve patients' long-term prognosis or reduce the risk of death within 1 month. These data suggest that central nervous system prophylaxis is necessary. The overall survival was longer than that in data from 1949 to 2009.
