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Treatment of Childhood Hypereosinophilic Syndrome with Mepolizumab
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A5576 - Treatment of Childhood Hypereosinophilic Syndrome with Mepolizumab
Author Block: S. Rose1, B. Wistinghausen2, C. Spencer1, A. Vicencio1; 1Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY, United States, 2Pediatrics, Mount Sinai, New York, NY, United States.
Introduction: Hypereosinophilic syndrome in childhood is rare. We present a 13-year-old male with severe-persistent asthma and hypereosinophilic syndrome, successfully treated with mepolizumab.
Case Description: AB was referred to our clinic for evaluation and management of severe persistent asthma. Over the preceding year and despite escalating asthma therapy including chronic oral steroids, symptoms of cough, wheeze and difficulty breathing progressed, and pulmonary function tests consistently demonstrated moderate-to-severe reversible obstruction. An extensive evaluation at an outside institution identified no alternate etiology for his symptoms. Notably, absolute eosinophil count was 3300/uL and 1525/mL on separate occasions, and flexible bronchoscopy demonstrated >50% eosinophils in lavage fluid. Evaluation for parasitic and rheumatologic disease was negative. He was treated with a 6-week tapering course of oral steroids, which resulted in control of symptoms and normalization of pulmonary function tests. Repeat bronchoscopy at our institution revealed 63% eosinophils in lavage fluid as well as marked eosinophil infiltration on biopsy of tracheal mucosa. Skin biopsy demonstrated eosinophilic infiltration, and he was diagnosed with hypereosinophilic syndrome. Further evaluation identified no clonal T-cell population or TKI mutations, and bone marrow biopsy showed an increased number of mature eosinophils and no underlying malignancy. Because high dose oral corticosteroid therapy could not be weaned without an immediate increase in symptoms and decrease in pulmonary function tests, we initiated mepolizumab at the standard dose of 100mg monthly. One month following his first injection, absolute eosinophil count decreased to 200/uL and spirometry normalized. AB has had 3 doses of mepolizumab to date. His oral steroids have been discontinued, his symptoms are well controlled, current eosinophil count is 0, and spirometry remains normal.
Discussion: Treatment for childhood hypereosinophilic syndrome, other than oral steroids, is not well established. This is one of very few reports documenting the successful use of mepolizumab for childhood hypereosinophilic syndrome, demonstrating a dramatic improvement in clinical, physiologic and laboratory parameters.
Author Block: S. Rose1, B. Wistinghausen2, C. Spencer1, A. Vicencio1; 1Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY, United States, 2Pediatrics, Mount Sinai, New York, NY, United States.
Introduction: Hypereosinophilic syndrome in childhood is rare. We present a 13-year-old male with severe-persistent asthma and hypereosinophilic syndrome, successfully treated with mepolizumab.
Case Description: AB was referred to our clinic for evaluation and management of severe persistent asthma. Over the preceding year and despite escalating asthma therapy including chronic oral steroids, symptoms of cough, wheeze and difficulty breathing progressed, and pulmonary function tests consistently demonstrated moderate-to-severe reversible obstruction. An extensive evaluation at an outside institution identified no alternate etiology for his symptoms. Notably, absolute eosinophil count was 3300/uL and 1525/mL on separate occasions, and flexible bronchoscopy demonstrated >50% eosinophils in lavage fluid. Evaluation for parasitic and rheumatologic disease was negative. He was treated with a 6-week tapering course of oral steroids, which resulted in control of symptoms and normalization of pulmonary function tests. Repeat bronchoscopy at our institution revealed 63% eosinophils in lavage fluid as well as marked eosinophil infiltration on biopsy of tracheal mucosa. Skin biopsy demonstrated eosinophilic infiltration, and he was diagnosed with hypereosinophilic syndrome. Further evaluation identified no clonal T-cell population or TKI mutations, and bone marrow biopsy showed an increased number of mature eosinophils and no underlying malignancy. Because high dose oral corticosteroid therapy could not be weaned without an immediate increase in symptoms and decrease in pulmonary function tests, we initiated mepolizumab at the standard dose of 100mg monthly. One month following his first injection, absolute eosinophil count decreased to 200/uL and spirometry normalized. AB has had 3 doses of mepolizumab to date. His oral steroids have been discontinued, his symptoms are well controlled, current eosinophil count is 0, and spirometry remains normal.
Discussion: Treatment for childhood hypereosinophilic syndrome, other than oral steroids, is not well established. This is one of very few reports documenting the successful use of mepolizumab for childhood hypereosinophilic syndrome, demonstrating a dramatic improvement in clinical, physiologic and laboratory parameters.
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