Recurrent Large Pericardial Effusion and Tamponade in a Young Patient with Erdheim Chester Syndrome: A Case Report and Review of the Syndrome-Juniper Publishers
JUNIPER PUBLISHERS - OPEN ACCESS JOURNAL OF CARDIOLOGY & CARDIOVASCULAR THERAPY Abstract In this report, we present a case of young male with Erdheim Chester Syndrome, who presented with dyspnea due to recurrent large pericardial effusion and impending tamponade. The patient underwent percutaneous pericardiocentesis and started on steroid, interferon alpha and immunosuppressive treatment with subjective improvement of his symptoms and rate of pericardial fluid re accumulation. This report will give an updated review of the syndrome. Introduction Erdheim Chester Disease (ECD) is a Non- Langerhans histiocytic disorder without a clear cut etiology [ 1 , 2 ]. Non- Langerhans histiocytic disorders are named as such due to the fact that the disorder or the cells affected are derived from cells otherthan that of Langerhans- typically of monocytic/ macrophagic origin [ 3 ]. Erdheim Chester Disease causes inflammation followed by invasion and ...