Response of idiopathic hypereosinophilic syndrome to treatment with imatinib mesylate.
Idiopathic hypereosinophilic syndrome (HES) is a rare hematologic disorder characterized by persistent eosinophilia with organ involvement. Patients with HES have a poor prognosis, but the disease course can be heterogeneous. Treatment of HES has included corticosteroids, chemotherapeutic agents such as cyclophosphamide, vincristine, hydroxyrea, and most recently interferon-alpha (IFN-alpha) which has shown long-term beneficial effects. We herein report on a patient with HES who had disease resistant to steroids, and chemotherapy with 2-chlorodeoxyadenosine and cytarabine, but who had a significant response after only 8 days of treatment with imatinib mesylate 100mg daily. The possible mechanism of response is discussed. This observation may lead to a better understanding of the pathophysiology of HES, and may provide a new form of effective therapy for the disease.
Department of Leukemia, M.D. Anderson Cancer Center, University of Texas, 1515 Holcombe Boulevard, Box 428, Houston, TX 77030, USA.
Patricia
P
Ault
Jorge
J
Cortes
Charles
C
Koller
Elizabeth S
ES
Kaled
Hagop
H
Kantarjian
D000477:Alkylating Agents / Q000627:therapeutic use; D000963:Antimetabolites / Q000627:therapeutic use; D001549:Benzamides; D001842:Bone and Bones / Q000098:blood supply; D002066:Busulfan / Q000627:therapeutic use; D017338:Cladribine / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003244:Consciousness Disorders / Q000209:etiology; D003561:Cytarabine / Q000627:therapeutic use; D018432:Drug Resistance, Multiple; D004791:Enzyme Inhibitors / Q000494:pharmacology / Q000627:therapeutic use*; D017809:Fatal Outcome; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D017681:Hypereosinophilic Syndrome / Q000150:complications / Q000188:drug therapy* / Q000601:surgery; D000068877:Imatinib Mesylate; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007213:Indomethacin / Q000627:therapeutic use; D007238:Infarction / Q000209:etiology; D008297:Male; D008875:Middle Aged; D008946:Mitral Valve Stenosis / Q000209:etiology; D010198:Pancytopenia / Q000209:etiology; D010879:Piperazines / Q000627:therapeutic use*; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors*; D011743:Pyrimidines / Q000627:therapeutic use*; D013156:Splenectomy
D000477:Alkylating Agents; D000963:Antimetabolites; D001549:Benzamides; D004791:Enzyme Inhibitors; D007166:Immunosuppressive Agents; D010879:Piperazines; D011743:Pyrimidines; D003561:Cytarabine; D017338:Cladribine; D000068877:Imatinib Mesylate; D011505:Protein-Tyrosine Kinases; D002066:Busulfan; D006918:Hydroxyurea; D007213:Indomethacin