X-linked agammaglobulinemia

X-linked agammaglobulinemia
X-linked infantile agammaglobulinemia a primary X-linked immunodeficiency disorder (gene locus: Xq21.3-q22) caused by mutation of the gene encoding Bruton tyrosine kinase. It is characterized by absence of circulating B lymphocytes, absence of plasma cells and germinal centers in lymphoid tissues, and very low levels of circulating immunoglobulins. The pathogenic defect appears to be a failure of pre-B cells to differentiate into mature B cells, express surface immunoglobulins, and produce antibody. Patients are unusually prone to bacterial infection and many have symptoms resembling those of rheumatoid arthritis. Called also Bruton a. or disease and X-linked hypogammaglobulinemia.

Medical dictionary. 2011.

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  • X-linked agammaglobulinemia — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 1728 ICD10 = ICD10|D|80|0|d|80 ICD9 = ICD9|279.04 ICDO = OMIM = 300300 MedlinePlus = eMedicineSubj = ped eMedicineTopic = 294 eMedicine mult = eMedicine2|derm|858 | MeshID = X linked… …   Wikipedia

  • agammaglobulinemia — Absence of, or extremely low levels of, the gamma fraction of serum globulin; sometimes used loosely to denote absence of immunoglobulins in general. SEE ALSO: hypogammaglobulinemia. acquired a. SYN: common variable immunodeficiency. Bruton a. an …   Medical dictionary

  • X-linked lymphoproliferative disease — Classification and external resources ICD 10 D82.3 OMIM 308240 300635 …   Wikipedia

  • Bruton agammaglobulinemia (disease) tyrosine kinase — Bru·ton agammaglobulinemia (disease), tyrosine kinase (brooґtən) [Col. Ogden Carr Bruton, American pediatrician, 1908–2003] see X linked agammaglobulinemia, under agammaglobulinemia, and see Bruton tyrosine kinase …   Medical dictionary

  • X-linked adrenal hypoplasia congenita — Classification and external resources OMIM 300200 DiseasesDB 32988 X linked adrenal hypoplasia congenita is a genetic dis …   Wikipedia

  • X-linked hypogammaglobulinemia — X linked infantile hypogammaglobulinemia see under agammaglobulinemia …   Medical dictionary

  • Bruton agammaglobulinemia — X linked a …   Medical dictionary

  • XLA — X linked agammaglobulinemia …   Medical dictionary

  • X-LA — X linked agammaglobulinemia …   Medical dictionary

  • Bruton disease — X linked agammaglobulinemia …   Medical dictionary

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