A 79-year-old man with primary chylopericardium associated with large granular lymphocytosis was followed for more than 26 years. Except for development of dyspnea on exertion during the past four years and more recently cough with sputum production, he has remained largely asymptomatic over this interval. Based on detailed examinations of cellular and humoral immunity, we speculate that increased natural killer cell activity and an increased number of large granular lymphocytes circulating in his peripheral blood represent a reactive response to production of various cytokines secondary to persistent loss (? chyloptysis) and sequestration of central lymph.
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