IDIOPATHIC THROMBOCYTOPENIC PURPURA IN EGYPTIAN CHILDREN

350 patients with idiopathic thrombocytopenic purpura (ITP) aged 2/12- 15 years (mean 6.3 +/- 2.7) were followed up during the period January Ist, 1975 to March 31, 1992. They constituted 40% of cases with hemor rhagic diathesis attending the Hematology/Oncology Clinic, Children's Hospital, Ain Shams University (relative frequency of 37.4/100.000 of the general Out-Patient Clinic in the same hospital). These patients p resented with acute (71.4%), chronic (22.9%) and recurrent (5.7%) form s. The age of presentation was younger in acute ITP. In the recurrent form there was significant female predominance. Most cases of acute IT P (66%) presented in winter and spring, with a positive history of pre ceding viral illness in 50% in contrast to 10% in chronic form. Four c hronic ITP cases developed lupus erythematosus; all were females > 9 y ears. As regards therapy, acute ITP cases with initial platelet count (PC) < 10 x 10(9)/I were randomized to receive either high-dose methyl prednisolone (HDMP) 10 mg/kg/day for 5 days i.v. (n = 10) or intraven ous immunoglobulin (IVIG) 0.4 g/kg/day for 5 days (n = 10) or conventi onal-dose prednisone (CDP) 2 mg/kg/day 4 weeks p.o. (n = 10). A dramat ic response was noticed in the first two groups. In chronic ITP, (n = 80) CDP induced complete response (CR) in 30% and partial response (PR ) in 20%; 50% were nonresponders. Twenty-four refractory ITP with pers istent PC less than or equal to 20 x 10(9)/I received second-line ther apy: vincristine 1.5 mg/m(2)/week i.v. 4 doses (n = 4) with no clinica l or hematological improvement. IVIG 0.4 g/kg/day for 5 days (n = 8) w ith sustained CR only in 2 patients (25%) and PR in 2 patients (25%). Splenectomy was performed (n = 12) with CR in 50%; out of them, 2 pati ents had shown no improvement on prior IVIG therapy. In conclusion, IT P is a benign condition with no fatality reported, but it could run a chronic refractory course.