Thrombotic thrombocytopenic purpura in childhood

A survey was conducted among 46 pediatric institutions in the United States and Canada to evaluate the presentation, therapy and survival of children with thrombocytopenic purpura (TTP) seen since 1960. TTP is an uncommon disease in the pediatric age group, and the clinical and pathologic findings in adults and children are almost identical. It can be distinguished from haemolytic uraemic syndrome in that it usually occurs in older children, the renal disease is milder, and the central nervous system symptoms appear to be related to vascular occlusive disease, and not to the complications of severely compromised kidneys. Appropriate biopsy specimens which reveal typical widespread hyaline occlusion of arterioles may confirm the diagnosis. Combinations of corticosteroids, splenectomy, and heparin have been used as therapy. Prognosis has improved, and is probably related to improved supportive care.