Beta-Blocker Therapy and Hemophagocytic Lymphohistiocytosis: A Case Report

[摘要] Objective. The aim of this paper is to describe a fatal case of hemophagocytic lymphohistiocytosis (HLH) in a patient withsevere heart failure, who was treated with low-dose propranolol.Patient and Interventions. We report on a 7-month-old boy withDowns syndrome who was born with an unbalanced, left dominant atrioventricular septal defect and aortic coarctation. Despitecoarctation repair and pulmonary artery banding he developed intractable heart failure and fever of unknown origin. Since heremained in heart failure he received a trial of low-dose propranolol to stabilize his cardiopulmonary status, which resulted in unexpected immunomodulatory effects.Measurements and Main Result. Immunoactivation was evidenced by high concentrationsof procalcitonin, soluble CD 25, tumor necrosis factorα, and interleukin 6 and 8. Propranolol resulting in hepatic compromiseas indicated by high lactate dehydrogenase and alanine aminotransferase levels. A therapeutic switch from propranolol to theβ1-receptor blocker metoprolol appeared to be instrumental in hemodynamic improvement and allowed discharge from hospital. However, the infant ultimately died from secondary inflammatory reactivation and intractable pulmonary obstructive disease. The autopsy results revealed HLH.Conclusion. Our case describes HLH secondary to heart failure and Downs syndrome. In this highly activated inflammatory state the beneficial hemodynamic effects of propranolol may be accompanied by immunomodulatory effects and the risk of acute liver failure. HLH occurs with a distinct pathophysiology, and specific treatment might be mandatory to increase the chance of survival.

[学科分类] 心脏病和心血管学