THE TERM idiopathic thrombocytopenic purpura (ITP) should be reserved for that hemorrhagic disorder characterized by a subnormal platelet count (usually below 50,000/cu mm) in the presence of a normal marrow containing normal or increased megakaryocytes and the absence of systemic disease capable of inducing thrombopenia. Bone marrow examination is mandatory to rule out leukemia, other infiltrative disorders, and hypoplastic and aplastic states; an L.E. preparation is indicated as are the careful search for systemic infection and renal disease and the detailed inquiry concerning drug ingestion. Although no specific antecedent event can be identified in most cases of ITP, it is recognized that some of the common childhood exanthemata may occasionally be followed by thrombocytopenic purpura (e.g., rubella, rubeola, varicella).
The rational approach to treatment must be based upon understanding of the natural history of the disease. Acute ITP has an excellent prognosis and approximately 80% affected children will make a complete and permanent recovery without specific therapy. Of these, three-quarters will recover within 3 months of onset, most within 4 to 6 weeks. Approximately 20% of cases will persist longer than 6 months and are then usually designated as chronic.
The mortality rate in acute ITP is extremely low and most of the urgency for treatment stems from concern over central nervous system hemorrhage. It seems clear that the incidence of CNS bleeding is no greater than 2-4% and that in most series reporting a greater incidence cases were not limited to ITP but included instances of thrombotic thrombocytopenic purpura and purpura fulminans, i.e., disease states associated with vasculitis.