X-linked lymphoproliferative syndrome

(redirected from 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.

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

a rare X-linked immunodeficiency in which there is a normal response to childhood infection but infection with Epstein-Barr produces a fatal lymphoproliferative disorder. Most patients die of acute infection. Others develop hypogammaglobulinemia, B-cell lymphoma, aplastic anemia, or agranulocytosis.

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 ?
He was just six when La Garde first tricked her factory worker husband into believing their son had been diagnosed with potentially fatal auto-immune lymphoproliferative syndrome - a serious and debilitating blood disorder.
Cancer of the prostate, lymphoproliferative syndrome, including such diseases as malignant lymphoma, chronic lymphocytic leukemia, Hodgkin's lymphoma, and non-Hodgkin's lymphoma
The day started with presentations on Autoimmune Lymphoproliferative Syndrome, ANCA and Quantiferon Gold before a well earned morning tea.
In all, 13 patients had primary (idiopathic) AIHA, whereas 2 had primary Evans Syndrome (ES), 2 had autoimmune lymphoproliferative syndrome (ALPS) + ES, and 1 had Wiskott-Aldrich syndrome (WAS) + AIHA.
For LYG, increased frequency is associated with both congenital and acquired immunodeficiency, such as X-linked lymphoproliferative syndrome, Wiskott-Aldrich syndrome, and HIV/ AIDS in which T-cell surveillance is deficient (8).
Recently, cases of sinus histiocytosis with massive lymphadenopathy associated with autoimmune lymphoproliferative syndrome have been described.
They even highlight the fact that failure of the normal apoptosis process plays a role in different diseases including cancer, certain neurodegenerative disorders such as Parkinson's and immune diseases, such as autoimmune lymphoproliferative syndrome (ALPS).
A rare entity called x-linked lymphoproliferative syndrome (XLPS), also known as Duncan's disease or Duncan's syndrome, results in an inability to effectively fight off EBV infection.
Joshua Hartley and his brothers, Nathan, 10, Daniel, eight, and Luke, four, were diagnosed with an extremely rare genetic defect known as X-Linked Lymphoproliferative Syndrome - or Duncan's Syndrome - in November last year.
The eight-year-old and his three brothers Joshua, 12, Nathan, 11 and four-year-old Luke were diagnosed with the extremely rare genetic defect, known as X-Linked Lymphoproliferative Syndrome, or Duncan's Syndrome, in November 2003.
Josh's dad David described the illness - also known as x-linked lymphoproliferative syndrome - as a 'timebomb' with sufferers rarely living into their teens.
Joshua, 12, Nathan, 10, Daniel, eight, and Luke Hartley, four, were diagnosed with the extremely rare genetic defect known as X-linked lymphoproliferative syndrome, or Duncan's syndrome, in November last year.