Clinical management of the hypereosinophilic syndromes.
Hypereosinophilic syndromes (HESs) are rare disorders characterized by marked hypereosinophilia that is directly responsible for organ damage or dysfunction. Different pathogenic mechanisms have been discovered in patient subgroups leading to the characterization of myeloproliferative and lymphocytic disease variants. In the updated terminology, idiopathic HES is now restricted to patients with HES of undetermined etiology. The practical clinical approach of patients with the different HES variants is reviewed herein, focusing on specific diagnostic tools and therapeutic options. Corticosteroids, hydroxyurea and IFN-α remain the classical agents for treatment of most patients with HESs. The specific role of therapeutic compounds that have become available more recently, namely, tyrosine kinase inhibitors and IL-5 antagonists, is discussed.
Department of Internal Medicine, Hôpital Erasme, Université Libre de Bruxelles, B-1070 Brussels, Belgium. ecogan@ulb.ac.be
Elie
E
Cogan
Florence
F
Roufosse
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000906:Antibodies / Q000627:therapeutic use; D001549:Benzamides; D004791:Enzyme Inhibitors / Q000627:therapeutic use*; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D017681:Hypereosinophilic Syndrome / Q000175:diagnosis* / Q000188:drug therapy* / Q000276:immunology / Q000601:surgery; D000068877:Imatinib Mesylate; D007155:Immunologic Factors / Q000627:therapeutic use*; D016898:Interferon-alpha / Q000627:therapeutic use*; D015848:Interleukin-5 / Q000276:immunology; D010879:Piperazines / Q000627:therapeutic use; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D033581:Stem Cell Transplantation
D000305:Adrenal Cortex Hormones; D000906:Antibodies; D001549:Benzamides; D004791:Enzyme Inhibitors; D007155:Immunologic Factors; D016898:Interferon-alpha; D015848:Interleukin-5; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D006918:Hydroxyurea