비장절제를 시행한 Wiskott-Aldrich 증후군 1례
- Author(s)
- 임상인; 박근수; 김명성; 이상락; 김흥식; 강진무
- Keimyung Author(s)
- Park, Geun Soo; Kim, Myung Sung; Lee, Sang Lak; Kim, Heung Sik; Kang, Chin Moo
- Department
- Dept. of Pediatrics (소아청소년학)
- Journal Title
- 대한혈액학회지
- Issued Date
- 1995
- Volume
- 30
- Issue
- 2
- Keyword
- Wiskott-Aldrich syndrome; Splenectomy
- 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.
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