Fulminant primary cardiac lymphoma with sudden cardiac death: A case report and brief review

• Published in: Journal of the Formosan Medical Association 2018-10, Vol.117 (10), p.939-943
• Main Authors: Cheng, Jen-Fang, Lee, Sze-Hwei, Hsu, Ron-Bin, Yu, Shan-Chi, Shun, Chia-Tung, Huang, Pang-Shuo, Chen, Ying-Hsien, Lee, Chii-Ming
• Format: Article
• Language: English
• Subjects: Cardiac; Death; Death, Sudden, Cardiac - etiology; Diagnosis, Differential; Echocardiography; Fatal Outcome; Heart neoplasms; Heart Neoplasms - diagnosis; Heart Neoplasms - pathology; Heart Neoplasms - surgery; Hemophagocytosis; Humans; Lymphoma; Lymphoma - diagnosis; Lymphoma - pathology; Lymphoma - surgery; Male; Middle Aged; Myocardium - pathology; Radiography, Thoracic; Risk Management; Sudden; Tomography, X-Ray Computed
• ISSN: 0929-6646; 1876-0821
• DOI: 10.1016/j.jfma.2018.03.011

Primary cardiac lymphoma (PCL) is very rare, with the variable clinical manifestations potentially leading to a delayed diagnosis. PCL is usually detected incidentally through image studies, whereas the diagnosis can be confirmed via analysis of pericardial effusion, endomyocardial biopsy tissue, or surgical specimens. Although no standard therapy has been established for PCL, without treatment, the prognosis is grave, with the estimated overall survival being approximately 1 year. We report a difficult diagnosis and complicated case of fulminant PCL, which is the first comprehensively reported case of PCL with secondary hemophagocytosis. A man presented with progressive dyspnea for 3 weeks, and then sudden cardiac death with ventricular fibrillation occurred. After resuscitation, echocardiography revealed a thickened left ventricular wall and severe mitral regurgitation, and computed tomography showed a right atrial mass with diffuse myocardial lesions. PCL was confirmed through a pathological analysis of specimens collected during mitral valvuloplasty, which also implied extensive myocardial involvement. Bone marrow biopsy demonstrated no evidence of lymphoma involvement, but secondary hemophagocytosis was noted. Despite aggressive chemotherapy, the patient died of sepsis with multiorgan failure 26 days after the operation.