Immunodeficiency: B Cell Development

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Immunodeficiency B Cell Development
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Brutons Agammaglobulinemia

• 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

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Brutons Agammaglobulinemia
Infections of mucus membranes Middle ear
(Otitis) Sinuses (Sinusitis) Eyes
(Conjuctivitis) Nose (Rhinitis) Lungs
(Pneumonia) Joints (Arthritis) Blood
stream Most common infectious bacteria
Streptococcus Pneumococcus Staphylococcus

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Btk and Tec Family Kinases
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Btk Family Kinases
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Severe Combined Immunodeficiency
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ADA Deficiency
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NADPH Oxidase
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Sites of CGDMutations
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CGD Neutrophil Respiratory Burst
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K Flux Responsible for Rise in Intravacuolar pH
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Complement Deficiency
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Terminal Complement