The use of the anti-malaria drug Fansidar® (pyrimethamine and sulphadoxine) in the treatment of a patient with autoimmune lymphoproliferative syndrome and Fas deficiency

被引:18
作者
Ten Bosch, JEV
Demanet, C
Balduck, N
Bakkus, MHC
De Raeve, H
Desprechins, B
Otten, J
Thielemans, K
机构
[1] Free Univ Brussels, Sch Med, Physiol Lab, B-1090 Brussels, Belgium
[2] Free Univ Brussels, Lab Haematol Immunol, Brussels, Belgium
[3] Free Univ Brussels, Childrens Hosp, Brussels, Belgium
[4] Hosp Vrije Univ Brussel, Dept Pathol, Brussels, Belgium
关键词
Fas deficiency; autoimmune lymphoproliferative syndrome; pyrimethamine; sulphadoxine; Fansidar;
D O I
10.1046/j.1365-2141.1998.00791.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Fas is a protein that plays a major role in the apoptotic mechanism of several cell types, including white blood cells (WBC). Mutations of the Fas gene in humans are known to lead to autoimmune lymphoproliferative syndrome (ALPS). Glucocorticoids or cytostatic drugs are sometimes used to treat the lymphoproliferation in these patients. When treated with the anti-malaria drug Fansidar(R), a patient with ALPS showed a marked shrinkage of the lymph node masses, decrease in peripheral blood lymphocytes (PBL) and an increase in neutrophil numbers. in addition, an increased Fas expression was seen on all types of leucocytes.
引用
收藏
页码:578 / 581
页数:4
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