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Hypereosinophilic Syndrome: The New Great Masquera ...
Hypereosinophilic Syndrome: The New Great Masquerader
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Pdf Summary
Hypereosinophilic syndrome (HES) is a rare group of disorders defined by persistent hypereosinophilia with end-organ damage. Because it can affect many organs and mimic other diseases, careful evaluation is needed to identify both the involved organs and the specific HES subtype, which guides prognosis and treatment. Corticosteroids remain the main initial therapy, though newer targeted treatments are increasingly used.<br /><br />This case involved an 18-year-old woman with no prior medical history who presented with rash, abdominal pain, chest pain, and marked leukocytosis. Initial workup at an outside hospital showed hilar adenopathy on CT and right-sided heart dilation with reduced ejection fraction on echocardiography. After transfer, additional history revealed recent urticaria and bronchitis, incidental leukocytosis under evaluation for possible lymphoma, and a family history of asthma.<br /><br />On exam she had tachycardia, edema, and decreased breath sounds. Laboratory tests showed WBC 26,000 with 42% eosinophils, elevated troponin, and elevated BNP. Cardiac MRI confirmed severe right ventricular dysfunction. Bone marrow biopsy showed 40% eosinophils without blasts. Right heart catheterization ruled out shunt and pulmonary hypertension. Infectious studies were negative, and lymph node biopsy showed only reactive changes, excluding malignancy.<br /><br />With high suspicion for HES with cardiac and systemic involvement, she was started on high-dose steroids, which rapidly reduced eosinophils and improved symptoms. She was discharged on prednisone and hydroxyurea with hematology follow-up. Despite this, she later had recurrent emergency visits for respiratory and chest symptoms and died 1.5 years after the initial hospitalization from cardiopulmonary arrest.
Asset Subtitle
Vijay Duggirala
Meta Tag
Author List
Evelyn Goodyear, Vijay Duggirala
Category
Clinical Vignettes
Concept
Idiopathic Hypereosinophilic Syndrome
Concept
Corticosteroids
Concept
Hypereosinophilia
Concept
End-organ Dysfunction
Concept
Clinical Evaluation
Distinguished
Non-Finalist
Presenter Organization
The Ohio State Wexner Medical Center
Presenting Author
Vijay Duggirala
Track
Adult
Keywords
hypereosinophilic syndrome
eosinophilia
end-organ damage
cardiac involvement
right ventricular dysfunction
corticosteroid therapy
hydroxyurea
leukocytosis
bone marrow biopsy
cardiopulmonary arrest
Idiopathic Hypereosinophilic Syndrome
Corticosteroids
Hypereosinophilia
End-organ Dysfunction
Clinical Evaluation
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