Hypereosinophilic syndrome: A multicenter, retrospective analysis of clinical characteristics and response to therapy

• Published in: Journal of allergy and clinical immunology Vol. 124; no. 6; pp. 1319 - 1325.e3
• Main Authors: Ogbogu, Princess U., MD, Bochner, Bruce S., MD, Butterfield, Joseph H., MD, Gleich, Gerald J., MD, Huss-Marp, Johannes, MD, Kahn, Jean Emmanuel, MD, Leiferman, Kristin M., MD, Nutman, Thomas B., MD, Pfab, Florian, MD, Ring, Johannes, MD, Rothenberg, Marc E., MD, PhD, Roufosse, Florence, MD, Sajous, Marie-Helene, MD, Sheikh, Javed, MD, Simon, Dagmar, MD, Simon, Hans-Uwe, MD, PhD, Stein, Miguel L., MD, Wardlaw, Andrew, MD, Weller, Peter F., MD, Klion, Amy D., MD
• Format: Journal Article
• Language: English
• Published: New York, NY Mosby, Inc 01.12.2009; Elsevier; Elsevier Limited
• Subjects: Adolescent; Adrenal Cortex Hormones - administration & dosage; Adrenal Cortex Hormones - therapeutic use; Adult; Aged; Aged, 80 and over; Allergy and Immunology; Antibodies, Monoclonal - administration & dosage; Antibodies, Monoclonal - therapeutic use; Antibodies, Monoclonal, Humanized; Benzamides; Biological and medical sciences; Chemokine CCL17 - blood; Chemokines; Child; Colleges & universities; Cyclosporine - administration & dosage; Cyclosporine - therapeutic use; Drug therapy; Drug Therapy, Combination; Eosinophil; Eosinophils - drug effects; Eosinophils - immunology; Eosinophils - metabolism; Female; FIP1L1-PDGFRA; Flow cytometry; Fundamental and applied biological sciences. Psychology; Fundamental immunology; Hematologic and hematopoietic diseases; Humans; Hydroxyurea - administration & dosage; Hydroxyurea - therapeutic use; hypereosinophilic syndrome; Hypereosinophilic Syndrome - drug therapy; Hypereosinophilic Syndrome - immunology; Hypereosinophilic Syndrome - metabolism; Imatinib Mesylate; Immunopathology; Interferon-alpha - administration & dosage; Interferon-alpha - therapeutic use; Interleukin-5 - blood; Ligands; Light therapy; Male; Medical sciences; Middle Aged; mRNA Cleavage and Polyadenylation Factors - immunology; mRNA Cleavage and Polyadenylation Factors - metabolism; Oncogene Proteins, Fusion - immunology; Oncogene Proteins, Fusion - metabolism; Other diseases. Hematologic involvement in other diseases; Parasitic diseases; Piperazines - administration & dosage; Piperazines - therapeutic use; Pyrimidines - administration & dosage; Pyrimidines - therapeutic use; Receptor, Platelet-Derived Growth Factor alpha - immunology; Receptor, Platelet-Derived Growth Factor alpha - metabolism; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Tryptases - blood; Young Adult; TCR; Fip1-like 1–platelet-derived growth factor receptor α; GM; FP; CEL; Eosinophil; FIP1L1-PDGFRA; T-cell receptor rearrangement PCR; L-HES; Chronic eosinophilic leukemia; Lymphocytic variant hypereosinophilic syndrome; hypereosinophilic syndrome; Geometric mean; Thymus and activation-related chemokine; HES; TARC; Immunopathology; Symptomatology; Platelet derived growth factor receptor A; Immunology; Treatment; Multicenter study; Granulocyte; Hemopathy; Retrospective; Hypereosinophilic syndrome

Background Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders defined by persistent blood eosinophilia ≥1.5 × 109 /L, absence of a secondary cause, and evidence of eosinophil-associated pathology. With the exception of a recent multicenter trial of mepolizumab (anti–IL-5 mAb...