thioguanine-anhydrous and Eosinophilia

Idiopathic hypereosinophilic syndrome (HES) is a rapidly progressive disease that can result in death within weeks to months of diagnosis in untreated patients. We report two pediatric cases of HES. One patient has remained clinically and hematologically stable for 9 years with vincristine and 6-thioguanine (6TG), and the other has received vincristine alone for 6 months with a good response. The use of long-term vincristine and 6TG or vincristine alone has not been previously reported in the management of HES in children. Vincristine administered every 3-4 weeks with or without 6TG can produce a reduction in the overall white cell count and improvement of symptoms of hypereosinophilia. Although the mortality of patients with HES is high, aggressive treatment can result in significant clinical benefit and improved prognosis in children with this syndrome.

Topics: Child, Preschool; Drug Therapy, Combination; Eosinophilia; Female; Humans; Syndrome; Thioguanine; Time Factors; Vincristine

In two of four patients with hypereosinophilia and Löffler's myocarditis (confirmed by biopsy) the activity of the disease was contained with prednisone, in one instance combined with hydroxycarbamide. In two patients, in whom the disease was taking a fulminant course and other treatment had failed, cytarabine and 6-thioguanine proved effective, providing a 16-month symptom-free period in one of them. But the second patient died from septicaemia associated with treatment-induced bone-marrow hypoplasia. Prednisone (1 mg/kg daily) and hydroxycarbamide (0.5-1.5 g daily) are the drugs of choice in the hypereosinophilia syndrome. If they fail, cytarabine (100 mg/m2 on days 1-5, repeated on day 28) and 6-thioguanine (100 mg/m2 daily) should be given.

Topics: Adult; Cytarabine; Endocarditis; Eosinophilia; Female; Humans; Hydroxyurea; Male; Middle Aged; Prednisone; Thioguanine

Topics: Adult; Cytarabine; Eosinophilia; Heart Failure; Humans; Hydroxyurea; Male; Prednisone; Syndrome; Thioguanine

Topics: Adolescent; Blood Cell Count; Bone Marrow Examination; Cytarabine; Eosinophilia; Female; Humans; Karyotyping; Leukemia, Lymphoid; Mercaptopurine; Mycoses; Sulfamethoxazole; Thioguanine; Trimethoprim; Vincristine