Table 1.

Diagnostic criteria for ALPS


Required 
   1. Chronic nonmalignant lymphoproliferation  
   2. Defective in vitro Fas-mediated lymphocyte apoptosis  
   3. At least 1% TCR α/β+, CD3+, CD4-, CD8- cells (DNTs) in peripheral blood or lymphoid tissue  
Supporting 
   1. Autoimmune antibodies  
   2. Mutations in Fas gene, Fas ligand gene, or caspase 8 or 10 genes
 
Adapted with permission from Bleesing et al.16  

Required 
   1. Chronic nonmalignant lymphoproliferation  
   2. Defective in vitro Fas-mediated lymphocyte apoptosis  
   3. At least 1% TCR α/β+, CD3+, CD4-, CD8- cells (DNTs) in peripheral blood or lymphoid tissue  
Supporting 
   1. Autoimmune antibodies  
   2. Mutations in Fas gene, Fas ligand gene, or caspase 8 or 10 genes
 
Adapted with permission from Bleesing et al.16  

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