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 ?
Castleman's disease (CD) is a rare lymphoproliferative syndrome typically involving a mediastinal mass.
This case described above is a difficult patient diagnosis in which the first clinical and imaging explorations (ultrasound and CT) guided toward a lymphoproliferative syndrome.
7%); autoimmune lymphoproliferative syndrome (10%); lipopolysaccharide-responsive beige-like anchor protein deficiency (10%); and DiGeorge syndrome (10%).
SHML has been found to occur concomitantly with other diseases such as diffuse large B-cell lymphoma, [1] HIV infection, [2] varicella zoster infection and autoimmune lymphoproliferative syndrome.
Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS).
Title: Post-Renal Transplant Lymphoproliferative Syndrome with Unique Lesion in Central Nervous System Treated with Radiotherapy: A Case Report and Literature Review
However, because of the relatively recent recognition of this spectrum, many names have been ascribed, including IgG4-related systemic disease, IgG4-related sclerosing disease, systemic IgG4 plasmacytic syndrome, and IgG4-related multiorgan lymphoproliferative syndrome.
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).