Rituximab treatment in refractory idiopathic thrombocytopenic purpura in children

Abstract

Sir: Children with refractory idiopathic thrombocytopenic purpura (ITP) present a therapeutic challenge. Due to the lack of controlled clinical trials, management of these patients is based on patient- and disease-related factors including platelet count, risk of bleeding and adverse effects of treatment. Pusiol et al. [1] reported two children successfully treated with rituximab. Based on reported findings, in 14-year-old girl presenting with repeated episodes of severe menorrhagia, most haematologists would consider laparoscopic splenectomy. In life-threatening intracranial haemorrhage of the same patient, assuming that conventional critical care measurements are already underway, appropriate management should include intravenous immunoglobulins (IVIg, 1 g/kg/day for 2 days), high-dose parenteral glucocorticoid (methylprednisolone 1 g/day for 3 days) and platelet transfusions [3]. In this report IVIg, documented previously to have prompt response, was not administered. Recombinant Factor VIIa, reported to be a very attractive alternative to control severe thrombocytopenic bleeding, was not considered. In addition, aminocaproic acid could be effective in such an emergency treatment. I report on 16-year-old girl suffering from chronic ITP refractory to corticosteroids, IVIg, and splenectomy. After two episodes of life-threatening bleeding (intracranial, and intraperitoneal from a ruptured corpus luteum), combined therapy consisting of rituximab and cyclosporine A was administered. The patient had no response to rituximab, despite a rapid and dramatic decrease in CD20+ cells. She is resistant to 6 months of cyclosporine A therapy. At present, chemotherapeutic drugs are being considered in this particularly serious situation.