Abstract

The management of chronic and refractory idiopathic thrombocytopenic purpura (ITP) in children is controversial. We conducted a retrospective review of our single center experience in China between 1990 and 2003 with splenectomy for chronic ITP in children in order to determine the initial and long-term hematological response, morbidity, mortality, predictors of response to splenectomy and the therapy in children who failed splenectomy. Of 65 children analyzed, the overall immediate clinical response to splenectomy was 89.2%. The median postsplenectomy follow-up time was 52 months (8–124). During follow-up, 9 children (13.8%) relapsed within a median time of 6 months (2–58). The overall morbidity was 1.5% and perioperative mortality was zero. During follow-up, 1 child died of intracranial hemorrhage (ICH) and 1 died of overwhelming postsplenectomy infection (OPSI). The platelet count at day 7 after splenectomy was a predictor of a sustained response to splenectomy but no preoperative parameters were predictors of the response to splenectomy. Of the 15 children who failed splenectomy, excluding the one who died of ICH, only 2 children intermittently required corticosteroids and IVIG. Splenectomy is a potential therapy to provide long-term control of disease in children with chronic ITP and is associated with low morbidity and mortality. The risk of fulminant sepsis remains an omnipresent concern. Antipneumococcal vaccination and antibiotic prophylaxis should be recommended and children should receive timely and adequate antibiotics for bacteria infection to lessen the problem of OPSI.

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