17 YEARS OF EXPERIENCE WITH CHRONIC IDIOPATHIC THROMBOCYTOPENIC PURPURA IN CHILDHOOD - IS THERAPY ALWAYS BETTER

Between 1975 and 1992 450 children with idiopathic thrombocytopenic pu rpura (ITP) were diagnosed, and of those 100 (22%) developed the chron ic form of the disease. Approximately half the patients with chronic I TP presented with mild to moderate hemorrhagic manifestations at the o nset of purpura (30 cases) and/or later during the course of the disea se (25 cases). The incidence of intracranial hemorrhage was 1%, and th e mortality rate due to overwhelming septicemia after splenectomy was also 1%. Overall one-third of the patients received no therapy; two-th irds of them went into spontaneous remission within 8 months to 8 year s from the onset of ITP. Steroids given in conventional or high doses (51 cases) achieved a transient (if any) rise in platelet count, but i n no case were steriods curative. Remission related to intravenous imm une globulin (IVIG) therapy was noticed in 38.5% of the children (10 o f 26) after variable courses. The response rate to splenectomy was 95. 0%. Ultimately the long-term outcome in children with chronic ITP was as follows: remission, 58 cases (spontaneous, 30; after IVIG therapy, 10; after splenectomy, 18); hemostatic platelet values, 22 cases (spon taneous, 16; after IVIG, 5; after splenectomy, 1). Thirteen children w ere lost in follow-up, and 7 remain thrombocytopenic but asymptomatic. These data indicate that chronic ITP in childhood runs a benign cours e in most cases and may remit with or without therapy even several yea rs from onset. Therefore, therapeutic intervention has to be individua lized, and splenectomy, which is not always safe, should be reserved f or problematic cases that fail to respond to conventional therapeutic modalities.