Stedman's Medical Dictionary
Stedman's > X-linked lymphoproliferative syndrome

X-linked lymphoproliferative syndrome

an X-linked recessive immunodeficiency and lymphoproliferative disease caused by mutation in the SH2 domain protein 1A gene (SH2D1A) on Xq; characterized by defective cellular or humoral immune response to Epstein-Barr virus; manifestations include fulminant infectious mononucleosis, B-cell malignancies, and hypogammaglobulinemia.

Synonym(s): Duncan disease, Duncan syndrome, lymphoproliferative syndrome, X-linked lymphoproliferative disease




 

 

 

 

 

 



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