Immune thrombocytopenic purpura of childhood

Abstract

Immune mediated thrombocytopenia (ITP) is a common manifestation of autoimmune disease in children. Although patients often present with bruises, petechiae, and some mucosal bleeding, the incidence of life-threatening hemorrhage is rare (0.2-0.9%) but can be fatal when presenting in vital organs. A wide range of therapeutic regimens are currently in use, including observation alone, as the majority of children recover within 4-6 months regardless of treatment. A growing understanding of the pathophysiology of acute ITP in children has not impacted the controversy surrounding treatment, but has clarified the mechanism of action of the most frequently used agents in chronic ITP. Newer monoclonal antibodies such as Rituxan have proved very useful in chronic or refractory ITP and studies are ongoing to determine the best regimens using this form of immune modulation. Splenectomy and newer agents to boost platelet production are also under study in chronic ITP. Neonates may also have a form of immune thrombocytopenia with extensive bruising and thrombocytopenia called neonatal alloimmune thrombocytopenic purpura (NATP). Rather than autoantibodies, the platelet destruction is secondary to transplacental maternal IgG alloantibodies. During pregnancy mothers may become sensitized to platelet membrane antigens present on fetal platelets. These antibodies may result in serious bleeding, including intracranial hemorrhage in the perinatal period. Once identified, these mothers may require treatment during future pregnancies to minimize serious bleeding in the fetus and neonate. Treatment in utero and immediately following delivery is focused on restoring neonatal platelets to a safe level and preventing life-threatening bleeding.