비장절제를 시행한 Wiskott-Aldrich 증후군 1례

Abstract

The Wiskott-Aldrich syndrome is a rare X-linked recessive disease characterized by thrombocytopenia, eczema and a marked vulnerability to recurrent infection. Patients with Wiskott-Aldrich syndrome have a poor antibody response to polysaccharide antigens, low levels of IgM and high levels of IgA and IgE in serum. Hemorrhages and infections are the main causes of infect mortality, whereas lymphoreticular malignancies are the main cause of death among adolescents. Although bone marrow transplantation of compatible bone marrow has been successful in some patients, suitable donors are not always available. Splenectomy produces a rapid and sustained rise in the platelet count, decreasing the risk of a major hemorrhage, but increasing the risk of overwhelming sepsis. Lifelong prophylaxis against fulminant infection is needed after splenectomy. We experienced a 10 year old boy with thrombocytopenia, eczema and a history of recurrent infection and immunodeficiency consistent with the Wiskott-Aldrich syndrome. The patient underwent splenectomy at 6 years of age because of recurrent hemorrhage. Postoperatively, his platelet count rose promptly and he had no further bleeding episodes.