Management of immune thrombocytopenic purpura in children

Immune thrombocytopenic purpura (ITP) is the most common acquired bleeding disorder occurring in previously healthy children and can be classified into two major forms. Acute and chronic ITP are benign conditions with a high probability of spontaneous recovery with or without therapy. Rates of 80-90% complete remission can be achieved irrespective of the treatment given. In only 10-20% of children thrombocytopenia persists for more than six months, showing a chronic course, which also has a high probability of remitting over time (up to 80% or more). The variability of the clinical course, and the lack of consistent clinical features, make the decision on whether and how to treat difficult. Most physicians are driven to treat all children with symptoms by concern over life-threatening hemorrhage, although the risk of intracranial hemorrhage (ICH) is only 0.1-0.9%. The commonly used treatment regimens for acute ITP are corticosteroids, intravenous immunoglobulins (IVIgG), or intravenous anti-D immunoglobulin (anti-D). So far, there is no evidence that initial therapy can prevent ICH or a chronic course of the disease. In chronic ITP the same drugs are generally used and it seems that pulses with steroids may be just as effective as IVIgG. Anti-D may also be considered a reliable and cheap alternative for chronic disease. A major problem in the management of chronic ITP is the question of whether repeated infusions of Ig (IVIgG or anti-D) and/or corticosteroids can postpone or ultimately preclude splenectomy, which must be considered only for a small proportion of patients resistant to therapy. In these cases, a laparoscopic approach should be preferred. Children who fail to respond to splenectomy (< 20% of cases) warrant second line treatment with other drugs, like cyclophosphamide or azathioprine and deserve a revisit of diagnosis for exclusion of secondary ITP.