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A1547 - Hypereosinophilic Syndrome with Eosinophilic Myocarditis
Author Block: J. Lam1, D. Patel1, R. Condos1, M. Mahmoudi2; 1Division of Pulmonary and Critical Care, NYU, New York, NY, United States, 2Division of Pulmonary and Critical Care, NYU, New York City, NY, United States.
Introduction
Hypereosinophilic syndrome (HES) is a rare condition that involves significant eosinophilia, end organ damage and possibly chronic irreversible fibrotic changes if not identified and treated appropriately. We report a case of HES in a previously healthy female who presented with multifocal pneumonia, elevated troponins and significant eosinophilia.
Case Presentation
Our patient is a 44 year old female born and raised in Nigeria until the age of 11 with no significant past medical history who presented with fevers, shortness of breath and cough. She reported sinus congestion and a sore throat about a month prior to admission. She traveled to Mexico for one week and upon return, developed fevers, worsening shortness of breath and nonproductive cough. Her admission imaging was significant for multifocal dense consolidations involving the right upper lobe and right lower lobe. Despite significant pulmonary involvement on imaging, she was in no respiratory distress and maintained normal oxygen saturations. Her blood work was notable for leukocytosis to 26.6 with 68% eosinophils and an absolute eosinophil count of 18,100. Extensive workup for infectious causes including helminths, parasites and fungi resulted negative. Bronchoscopic evaluation was significant for eosinophilia to 92% on bronchial alveolar lavage, and bone marrow biopsy demonstrated predominantly eosinophils at all stages of maturation consistent with HES. Her presentation was further complicated by up-trending troponins concerning for eosinophilic myocarditis. She did not have a history of cardiac disease and was chest pain free throughout. The patient was initiated on prednisone, and showed significant improvement in eosinophilia within 24 hours. On outpatient follow up, the patient continued to show excellent response to prednisone, with complete resolution of symptoms and normalization of white blood cells, eosinophils, troponins, and infiltrates on imaging. Her PDGFRA gene studies resulted negative, so there was no indication for concomitant imatinib therapy.
Discussion
Patients with hypereosinophilic syndrome have end organ injury typically involving the lungs, heart and intestines. The lungs are involved in 25% of cases but the most significant morbidity and mortality tends to result from cardiac involvement. Patients with cardiac involvement are at risk for developing congestive heart failure, cardiomyopathy and chronic fibrosis if not detected and treated. Early detection and treatment including exclusion of potential secondary causes of eosinophilia has been vital in decreasing the mortality since the disease was initially described over 40 years ago.