Immunodeficiency Agammaglobulinemia

Primary immunodeficiency diseases (PIDs) are defects of the immune system that are due to genetic abnormalities or some failure in normal embryological development. They are usually apparent at birth or develop shortly thereafter. Approximately 70 PIDs have been described, including those specihc for humoral immunity (e.g., X-linked agammaglobulinemia, immune globulin [Ig] A dehciency), cellular immunity (e.g., DiGeorge s syndrome), or both (e.g., severe combined immunodehciency syndrome). 

Immune globulin, given intramuscularly or intravenously, is recommended in the treatment of primary humoral immunodeficiency, congenital agammaglobulinemias, common variable immunodeficiency, severe combined immunodeficiency, idiopathic thrombocytopenic purpura, and autoimmune hemolytic anemia. There are six licensed preparations of immune globulin. 

Primary immunodeficiency disorders Immune globulin (intravenous)2 Consult the manufacturer s dosing recommendations for the specific product being used. Primary immunodeficiency disorders include specific antibody deficiencies (eg, X-l inked agammaglobulinemia) and combined deficiencies (eg, severe combined immunodeficiencies). 

Intravenous immunoglobulin (IVIC) In recent years indications for the use of intravenous immunoglobulin (IVIG) have expanded beyond replacement therapy for agammaglobulinemia and other immunodeficiencies to... 

Agammaglobulinemia Primary immunodeficiency disease caused by failure of B cells to develop, resulting in lack of antibodies. 

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