Efficacy and Safety of Mepolizumab (Anti-Interleukin-5) Treatment in Gleich’s Syndrome
| Autor: | Francesco Annunziato, Sara Pratesi, Andrea Matucci, Alessandra Vultaggio, Laura Dies, Marie-Pierre Piccinni, Enrico Maggi, Francesco Liotta, Francesca Nencini, Emanuele Vivarelli |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
lcsh:Immunologic diseases. Allergy
Adult Male medicine.medical_specialty Exacerbation Fever Population Immunology Hypereosinophilia Case Report 030204 cardiovascular system & hematology Antibodies Monoclonal Humanized Gastroenterology 03 medical and health sciences Leukocyte Count 0302 clinical medicine Prednisone Internal medicine Hypereosinophilic Syndrome medicine Immunology and Allergy Humans 030212 general & internal medicine education Interleukin 5 hypereosinophilia anti-interleukin-5 Eosinophil cationic protein education.field_of_study business.industry angioedema mepolizumab medicine.disease Angioedema Anti-interleukin-5 Biological agents Gleich's syndrome Mepolizumab Gleich’s syndrome Immunoglobulin M biological agents medicine.symptom Interleukin-5 lcsh:RC581-607 business medicine.drug |
| Zdroj: | Frontiers in Immunology Frontiers in Immunology, Vol 9 (2018) |
| ISSN: | 1664-3224 |
| Popis: | Gleich’s syndrome (GS) is characterized by recurrent episodes of angioedema, increase in body weight, fever, hypereosinophilia, and elevated serum IgM. The exact etiology remains unclear. Currently, the only treatment strategy is the administration of high dose of steroids during the acute phases. We report the case of a 37-year-old man suffering from GS with recurrent episodes of angioedema, fever, hypereosinophilia [6,000/mm3 (45%)], and high eosinophil cationic protein (ECP) (>200 μg/l), treated with oral steroids during the acute phase (prednisone 50–75 mg/day), the dose of maintenance being 25 mg/day. No monoclonal components were identified, and genetic tests exclude mutations including Bcr/Abl, JAK2 V617F, c-KIT D816V, and FIP1L1-PDGFRA. Using Luminex technology, we observed higher serum levels of interleukin (IL)-5, CCL2, and CCL11 during the acute exacerbations in comparison with the clinical remission phases though CCL11 did not achieve statistical significance. The flow-cytometric analysis identified a CD3+ CD8− lymphocyte population with high frequency of IL-4-, IL-5-, and IL-13-producing cells. No clinical benefit was observed after therapeutic strategies with imatinib, interferon-α, cyclosporine-A, and azathioprine. Due to high IL-5 serum levels, an intravenous treatment with anti-IL-5 monoclonal antibody mepolizumab (750 mg every 4 weeks) was started. A reduction in the rate of exacerbation phases/year (10 ± 3 vs 2 ± 1; p |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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