X-linked lymphoproliferative syndrome

Type:Term

Definitions
1. 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.

Synonyms
Duncan disease , Duncan syndrome , lymphoproliferative syndrome , X-linked lymphoproliferative disease

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