Table 4.

Diagnostic criteria for XLP


Definitive XLP  

Male patients with lymphoma/Hodgkin disease (HD), fatal EBV infection, immunodeficiency, aplastic anemia or lymphohistiocytic disorder who have a mutation in SAP SH2D1A.  
Probable XLP   Male patients experiencing death, lymphoma/HD, immunodeficiency, aplastic anemia, or lymphohistiocytic disorder following acute EBV infection and maternal cousins, uncles, or nephews with a history of a similar diagnosis following acute EBV infection.  
Possible XLP
 
Male patients experiencing death, lymphoma/HD, immunodeficiency, aplastic anemia, or lymphohistiocytic disorder following acute EBV infection; this is sometimes described as sporadic XLP.
 

Definitive XLP  

Male patients with lymphoma/Hodgkin disease (HD), fatal EBV infection, immunodeficiency, aplastic anemia or lymphohistiocytic disorder who have a mutation in SAP SH2D1A.  
Probable XLP   Male patients experiencing death, lymphoma/HD, immunodeficiency, aplastic anemia, or lymphohistiocytic disorder following acute EBV infection and maternal cousins, uncles, or nephews with a history of a similar diagnosis following acute EBV infection.  
Possible XLP
 
Male patients experiencing death, lymphoma/HD, immunodeficiency, aplastic anemia, or lymphohistiocytic disorder following acute EBV infection; this is sometimes described as sporadic XLP.
 

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