文档介绍：Immunodeficiency: B Cell Development
First described in 1952 by Dr Ogden Bruton
-Bruton's Agammaglobulinemia
-Congenital Agammaglobulinemia
Inherited immunodeficiency male disease in which patients lack the ability to produce
-Antibodies
-Proteins that make up gamma globulin
-Immunoglobulin fraction of blood plasma
Affected males
Few or no B cells and lymph nodes are very small & No tonsils and cervical lymph nodes
Serum
Usually no IgA, IgM, IgD, or IgE; small amounts of IgG from placenta
First 6-12 months
Protection by maternal IgG from placenta to fetus
As IgG is exhausted
Males develop recurrent pyogenic infections
Bruton’s Agammaglobulinemia
Infections of mucus membranes
* Middle ear (Otitis)
* Sinuses (Sinusitis)
* Eyes (Conjuctivitis)
* Nose (Rhinitis)
* Lungs (Pneumonia)
* Joints (Arthritis)
* Blood stream
* mon infectious bacteria
* us
* us
* us
Bruton’s Agammaglobulinemia
Btk and Tec Family Kinases
Btk Family Kinases
bined Immunodeficiency