Approach to the investigation and management of immune thrombocytopenic purpura in children.

摘要: Childhood immune thrombocytopenic purpura (ITP) is typically a benign, self-limiting disorder occurring in young (<10 years of age) previously healthy children. More than 80% such children enter complete sustained remission within few weeks to months initial presentation, irrespective any therapy given. The major concern the small but finite (0.1 0.9%) risk intracranial hemorrhage, which occurs with very low platelet counts (<20 × 109/L), and justification for treatment increase circulating count. Effective strategies are single-dose intravenous immunoglobulin G (IVIgG; ~1 g/kg) medium high-dose corticosteroids, administered orally or parenterally. necessity bone marrow aspiration hospitalization continues be debated. In chronic ITP, defined by persistence thrombocytopenia ≥6 months, splenectomy should considered relatively subgroup symptomatic, severe who have either failed an adequate trial (≥12 months) primary (IVIgG, anti-D, corticosteroids) intolerant therapy. Laparoscopic preferred over open splenectomy. Children fail respond (≤20% cases) evaluated presence accessory spleens; their management often difficult must individualized. refractory cases, second-line therapies (such as azathioprine vinca alkaloids) need considered. Secondary ITP rare sometimes associated other autoimmune cytopenias (Evan's syndrome, neutropenia). These cases poorly conventional medical response rates considerably lower ITP.