X-linked agammaglobulinemia

Agammaglobulinemia is a deficiency disease in which the body does not form specific antibodies.

Agammaglobulinämien in humans

There are different forms of agammaglobulinemia. All forms are characterized by an immunodeficiency in the form of lack of antibodies.

  • The Bruton 's syndrome ( XLA ) occurs in newborns. It is inherited as an X -linked recessive.
  • The Autosomal recessive agammaglobulinemia ( ARA) is the clinical appearance comparable according to the Bruton 's syndrome, but is autosomal. Causes mutations in at least two genes, and IGHM IGLL1 encoding the Ig subunit mu and lambda- like-1.
  • The secondary agammaglobulinemia, which occurs as a result of a general severe protein deficiency or as part of a severe combined immunodeficiency are acquired in contrast to the first two and not inherited.

Agammaglobulinämien in pets

Horses: In Arabian foal may occur agammaglobulinemia by an autosomal recessive combined immunodeficiency (CID ). This lack both the globulinproduzierenden B- lymphocytes as well as those responsible for cellular defense T lymphocytes. Three horses have been observed worldwide, which had a agammaglobulinemia as a result of the selective absence of B - lymphocytes.

Dogs: So far, an isolated case of CID was known, who appeared in a family of Bassethunden. A more severe form of the CID also occurs with Jack Russell Terriers.

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