Primary immunodeficiency : X-linked agammaglobulinemia

What is X-linked agammaglobulinemia.

X-linked agammaglobulinemia (XLA) was first described in 1952 by Dr. Ogden Bruton.This disease, sometimes called Bruton's agammaglobulinemia or congenital agammaglobulinemia, was one of the first detection of immunodeficiency diseases.

XLA is an inherited disease in which the patient's body can not produce antibodies, proteins that make up the gamma globulin or immunoglobulin fraction of blood plasma.

Antibodies are an integral part of the defense mechanism against certain microorganisms (such as bacteria and viruses).Antibodies are needed for recovery from infections.They also protect against getting certain infections.

There are antibodies specifically designed to connect to a specific microorganism - it looks like a matching key lock.When microorganisms such as bacteria, reach the mucous membranes or inside the body, a molecule of antibody specific to the microorganism adhere to its surface.

Attaching antibodies to the surface of the microorganism may have on

e or more effects that are useful for humans.For example, some microorganisms must attach to cells in the body to cause an infection, and antibodies do not allow microorganisms to adhere to the cells.

antibodies attached to the surface of some microorganisms and activate other protective forces of the body (for example, a group of blood proteins called serum komple-ment), which can directly destroy bacteria or viruses.Finally, antibody coated bacteria are much easier to ingest and kill white blood cells (phagocytes) than the bacterium not coated with antibodies.All of these actions prevent microorganisms from invading body tissues where they may cause serious infections.

basic defect in XLA is an inability of the patient to produce antibodies.Antibody - a protein produced by cells called plasma cells.The development of plasma cells
being in a particular order, starting from the stem cells located in the bone marrow.Stem cells are formed from immature lymphocytes called pro-B-cells.Pro-B cells are the precursors of pre-B cells, of which in turn formed B-lymphocytes.

Each B-lymphocyte bears on its surface immunoglobulin pattern that the cell can produce.This cell surface immunoglobulin can bind foreign substances called antigens.When the B-lymphocyte is contacted with the specific antigen, such as the pneumococcus or tetanus toxoid, it matures into plasma cells secreting antibodies.

Each B cell produces an antibody (immunoglobulin), slightly different from other cell products that allow the body to respond to millions of different foreign substances.Most patients with XLA have B-lymphocyte precursors, but very few of them go on to become B-lymphocytes.

As a result, when XLA primary defect is the inability of B-lymphocyte precursors to mature into B-cells.Patients with XLA have mutations in a gene required for the normal development of B-lymphocytes.This gene, discovered in 1993, called BTK (Bruton's tyrosine kinase gene) in honor of the discoverer of the disorder - Colonel, Dr. Ogden Bruton medicine.As the name of this disorder, BTK gene is located on the X chromosome.

Article courtesy of a worldwide organization IPOPI, working to improve the lives of people with primary immunodeficiency.
Copyright 2007 owned fund Immune Deficiency Foundation, USA."Guidelines for the primary immunodeficiency diseases for patients and their families", from which the material is taken under the license, was developed Immune Deficiency Foundation with support from Baxter Healthcare Corporation.

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