title: Efficacy of imatinib mesylate in a patient with idiopathic hypereosinophilic syndrome and severe heart involvement.
abstract: Idiopathic hypereosinophilic syndrome (HES) is a rare, chronic hematological disease mainly characterized by unexplained prolonged eosinophilia, with frequent evidence of secondary organ damage. Treatment with steroids, chemotherapy, interferon-alpha (IFN-alpha), or imatinib-mesylate may improve the prognosis. Here we describe the case of a young male patient with a six-year history of HES and severe heart involvement who, after unsuccessful treatment attempts with steroids, hydroxyurea and IFN-alpha, had a prompt, clinical and hematological complete remission following administration of imatinib. As his cardiac function also markedly improved, he was considered for heart transplant. However, seven years after the onset of the disease and four months after the termination of imatinib treatment the patient died of a cerebral hemorrhage that occurred during an episode of acute respiratory sepsis. Imatinib has been previously reported to be effective in some hematological conditions with no evidence of the BCR/ABL transcript. The mechanisms that are probably involved in the response to imatinib in HES are also discussed.
authors.affiliation: Hematology and Bone Marrow Transplantation Unit, S Camillo-Forlanini Hospital, Rome, Italy. g.anghel@quipo.it
authors.forename: Gabriel
authors.initials: G
authors.lastname: Anghel
authors.forename: Luca
authors.initials: L
authors.lastname: De Rosa
authors.forename: Carla
authors.initials: C
authors.lastname: Ruscio
authors.forename: Nicola
authors.initials: N
authors.lastname: Petti
authors.forename: Massimo
authors.initials: M
authors.lastname: Riccardi
authors.forename: Alessandro
authors.initials: A
authors.lastname: Severino
authors.forename: Ignazio
authors.initials: I
authors.lastname: Majolino
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides; D004334:Drug Administration Schedule; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D017809:Fatal Outcome; D006331:Heart Diseases / Q000188:drug therapy*; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000188:drug therapy*; D000068877:Imatinib Mesylate; D008297:Male; D010879:Piperazines / Q000008:administration & dosage / Q000627:therapeutic use*; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D011743:Pyrimidines / Q000008:administration & dosage / Q000627:therapeutic use*; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D004791:Enzyme Inhibitors; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D011505:Protein-Tyrosine Kinases