X-linked lymphoproliferative syndrome


Also found in: Acronyms.

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.

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.

X-link·ed lym·pho·pro·lif·er·a·tive syn·drome

(lingkt lim'fō-prō-lif'ĕr-ă-tiv sin'drōm)
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.
References in periodicals archive ?
All four children have X-Linked Lymphoproliferative Syndrome (XLP), a genetic disorder found in just 100 families worldwide since it was first identified in 1999.
HLH may also occur as a complication of Chediak-Higashi syndrome (35) or after EBV infection in patients with X-linked lymphoproliferative syndrome (36).
It was another cruel blow for the couple from Swinton, Manchester, who face losing three of their sons through the inherited genetic disorder, X-linked LymphoProliferative Syndrome.

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