Management of hypereosinophilic syndrome: a prospective study in the era of molecular genetics
| Autor: | Stavroula Giannouli, Michael Voulgarelis, Issa J Dahabreh, Christine Zoi, Haralampos M. Moutsopoulos, Katerina Zoi |
|---|---|
| Rok vydání: | 2007 |
| Předmět: |
Adult
Male medicine.medical_specialty Myeloid Receptor Platelet-Derived Growth Factor alpha Oncogene Proteins Fusion Population Antineoplastic Agents Piperazines Translocation Genetic Therapeutic approach Internal medicine Antineoplastic Combined Chemotherapy Protocols Hypereosinophilic Syndrome medicine Eosinophilia Humans Hydroxyurea Prospective Studies Prospective cohort study education reproductive and urinary physiology Aged mRNA Cleavage and Polyadenylation Factors education.field_of_study business.industry Hypereosinophilic syndrome Patient Selection Imatinib General Medicine Middle Aged medicine.disease medicine.anatomical_structure Pyrimidines Immunology Benzamides Cytogenetic Analysis Imatinib Mesylate Prednisone Female medicine.symptom business Serositis medicine.drug |
| Zdroj: | Medicine. 86(6) |
| ISSN: | 0025-7974 |
| Popis: | Hypereosinophilic syndrome (HES) is a heterogeneous group of disorders characterized by unexplained persistent primary eosinophilia causing end-organ damage. We conducted a prospective cohort study of patients fulfilling the diagnostic criteria for HES. Of 20 patients considered eligible for the study, 2 were found to have clonal myeloid disorders, limiting the diagnosis of "true" HES to 18 patients. No patient carried the FIP1L1-PDGFRA fusion gene or other imatinib-responsive translocations. A clonal interleukin-5-producing T-cell population was not detected in any patient. Common manifestations at presentation were pulmonary, cutaneous, and neurologic involvement; serositis; and gastrointestinal involvement. Only 3 patients developed cardiac involvement. Fifteen of the HES patients were administered first-line combined treatment with steroids and hydroxyurea. Nine patients achieved complete response, while 6 attained only partial response. Imatinib was administered to 3 HES patients who had been pretreated with steroids, resulting in complete hematologic and clinical response in 2 patients and no response at all in 1. Further treatment of the latter patient with steroids and hydroxyurea also proved ineffective. We conclude that the therapeutic approach should be individualized according to molecular findings. We consider the coadministration of corticosteroids and hydroxyurea to be an effective combination for the treatment of FIP1L1-PDGFRA-negative HES. |
| Databáze: | OpenAIRE |
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