A 41‐year‐old man with no medical history presented for a second opinion for chest pain, cough, fevers, and an 80‐pound weight loss in 2 months. Previous evaluation revealed peripheral eosinophilia, pericarditis, and pneumonia. On presentation to our facility, his WBC count was 28,000/μL with an eosinophil count of 12,600 cells/μL. A workup was performed to rule out secondary causes of hypereosinophilia including ANA, ANCA, cortisol‐stimulation testing, stool cultures, comprehensive ova and parasite testing, strongyloides, histoplasmosis and toxacara serologies, HIV, LDH, IgE, and an EGD . All were nondiagnostic. PFTs showed moderate restriction with decreased diffusion capacity. Bronchioalveolar lavage showed increased eosinophils (18%) with cultures negative. Cardiac MRI revealed an apical thrombus with subendocardial fibrosis suggesting eosinophilic myocarditis. Ejection fraction was mildly reduced at 45%. Bone marrow biopsy was performed, which revealed hypercellular marrow with 34% eosinophils and no blasts present. Testing for FIP1L1‐PDGFRA was negative. He was diagnosed with idiopathic hypereosinophiliac syndrome (HES) complicated by Loeffler's endocarditits (eosinophilic myocarditis) and restrictive lung disease. The patient was treated with anticoagulation, prednisone, and hydroxyurea. On follow‐up he had a reduction in his eosinophil count, relative symptom improvement, and full ejection fraction recovery.
Eosinophilia is a common finding in hospital medicine. However, a diagnosis of HES should always be considered for all patients with severe hypereosinophilia (>5000 cells/μL). HES is a heterogeneous group of disorders whose diagnostic criteria include (1) an eosinophil count of more than 1500, (2) no other evident cause for eosinophilia (allergic, adrenal, infectious, or malignant), and (3) signs or symptoms of organ involvement. Cardiac involvement including Loeffler's syndrome (which is eosinophilic subendocardial necrosis, thrombosis, and fibrosis) is common in HES and is a major cause of morbidity and mortality. Patients with organ involvement such as Loeffler's endocarditis and/or pulmonary disease should be started on immediate therapy to prevent irreversible damage. Diagnosis of specific subtypes of HES is crucial to determining therapy, as they respond differently to treatments. B12 and tryptase levels in addition to bone marrow morphologic, cytogenetic, immunophenoytpic, and molecular analysis (especially for the FIP1L1‐PDGFRA gene) help to diagnose HES subtypes. Treatments for different subtypes of HES include steroids, hydroxyurea, imatinib, and TNF alpha.
Although HES is rare, hospitalists should consider the diagnosis in patients with significant eosinophilia. Early diagnosis and determination of the specific subtype, followed by appropriate treatment may prevent irreversible organ damage.
To cite this abstract:Heacock A, Saultz J, Taylor M. Hypereosinophilic Syndrome: A Case Report of the Rare Cause of Cardiac and Pulmonary Disease in a 40‐Year‐Old Man. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 318. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/hypereosinophilic-syndrome-a-case-report-of-the-rare-cause-of-cardiac-and-pulmonary-disease-in-a-40yearold-man/. Accessed September 16, 2019.