agammaglobulinemia


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agammaglobulinemia

[ā‚gam·ə′gläb·yə·lən·ē·mē·ə]
(medicine)
The condition characterized by lack of or extremely low levels of gamma globulin in the blood, together with defective antibody production and frequent infections; primary agammaglobulinemia occurs in three clinical forms: congenital, acquired, and transient.
References in periodicals archive ?
X-linked agammaglobulinemia (XLA) / hypogammaglobulinemia, also known as Bruton's agammaglobulinemia is a prototype of humoral immunodeficiency first described by Bruton in 1952.
As mentioned, CVID may include deficiencies in other immunoglobulins as well, such as IgA and IgM deficiencies, although these deficiencies are more frequently associated with a group of other primary immunoglobulin deficiencies like agammaglobulinemia and Severe Combined Immunodeficiency (SCID).
15) XLA is an agammaglobulinemia disease in male patients with reduced number of B cells (< 1%), which is caused by mutations in bruton tyrosin kinase (BTK) gene.
Among the possible treatments are the elimination of chronic sinusitis, avoidance of or desensitization to offending allergens, repair of a cleft palate, treatment of agammaglobulinemia, or tonsillectomy and adenoidectomy.
David contracted VAPP when he was five-months old and remained undiagnosed for two years, when it was also found that he had Brutons agammaglobulinemia, a rare genetic disorder, which causes immune deficiency.
This includes, but is not limited to, common variable immunodeficiency (CVID), X-linked agammaglobulinemia, congenital agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies.
Neutropenia is observed with a rate of of 25% in X-linked agammaglobulinemia and with a rate of 50% in X-linked hyperimmune M syndrome.
3 Good syndrome is among the long list of immunodeficiency differentials which include immunodeficiency due toHIV/AIDS, X linked agammaglobulinaemia (XLA), Autosomal Recessive Agammaglobulinemia, Selective IgM Deficiency, Selective IgE Deficiency, Antibody Deficiency with Transcobalamin II Deficiency, Drug-Induced Antibody Deficiency, Interferon-a/IL-12 Pathway Deficiencies, Natural Killer Cell Deficiency, Phagocyte Killing Defects, Specific Granule Deficiency.
Octagam is indicated for treatment of primary humoral immunodeficiency (such as congenital agammaglobulinemia), common variable immunodeficiency, X-linked agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies.
Too little Btk causes a disease called Bruton's agammaglobulinemia, in which the B-lymphocytes fail to mature and produce antibodies, leading to infections.