Recurrent idiopathic thrombocytopenic purpura in childhood

Background. Idiopathic thrombocytopenic purpura (ITP) is a common haematological disease during childhood, that usually has a benign course; however, literature on the recurrent form of the disease (rITP) is limited. Procedure. rITP was characterized by intermittent episodes of thrombocytopenia (TP) followed by periods of recovery, unrelated to therapeutic: intervention. We retrospectively reviewed features of patients with rITP, diagnosed and systematically followed up at our center, during the period 1975-2004. Results. Forty-eight of 795 children with ITP (6.0%) presented with rITP. The majority of patients (68.8%) had only one recurrence, whereas only one patient had four. A time interval between two episodes longer than 3 months (up to 96) was identified in 2/3 of episodes and <3 months in 1/3. The initial episode and the first recurrence mostly shared features of acute ITP; however, 22.9% of the episodes appeared with a chronic self-limited course. Bleeding manifestations were rare (18.6% of episodes) and mild, and they tended to occur in severely thrombocytopenic patients, mainly at the onset of the initial episode; intracranial hemorrhage (ICH) occurred in a toddler with short duration thrombocytopenia. intravenous gamma globulin (IVIG) or corticosteroids were administered in 24.5% of episodes. None of the patients needed splenectomy. Conclusion. rITP is a rare, mild, self-limited type of ITP, although ICH may occur in a profoundly TP child. Recurrence may occur close or far apart to a previous isolated TP episode. The duration of episodes varies considerably from patient to patient and from episode to episode in the same patient. The pathogenesis of rITP still remains unclear.