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What are the effects of agammaglobulinemia?
X-linked agammaglobulinemia (a-gam-uh-glob-u-lih-NEE-me-uh) — also called XLA — is an inherited (genetic) immune system disorder that reduces your ability to fight infections. People with XLA might get infections of the inner ear, sinuses, respiratory tract, bloodstream and internal organs.
What causes XLA?
Frequently called Bruton’s Agammaglobulinemia, XLA is caused by a genetic mistake in a gene called Bruton’s tyrosine kinase (BTK), which prevents B cells from developing normally. B cells are responsible for producing the antibodies that the immune system relies on to fight off infection.
How is XLA treated?
Medications to treat XLA include:
- Gammaglobulin. This is a type of protein found in blood that contains antibodies against infections. It’s given by infusion into a vein every two to four weeks or by weekly injection.
- Antibiotics. Some people with XLA receive continuous antibiotics to prevent infections.
How common is XLA?
XLA occurs in approximately 1 in 200,000 newborns.
Is XLA a PID?
XLA is PID caused by Bruton’s tyrosine kinase gene mutation. XLA is characterized by virtual absence of all immunoglobulin isotypes and of circulating B lymphocytes.
What are the major symptoms of agammaglobulinemia?
Symptoms include frequent episodes of:
- Bronchitis (airway infection)
- Chronic diarrhea.
- Conjunctivitis (eye infection)
- Otitis media (middle ear infection)
- Pneumonia (lung infection)
- Sinusitis (sinus infection)
- Skin infections.
- Upper respiratory tract infections.
How can XLA be prevented?
Preventing bacterial infections is very important for people with XLA. Gammaglobulin (a type of protein in the blood that contains antibodies to prevent or fight infections) is the main treatment for people with XLA. In the past, most people received this by intravenous (IV) infusion every two to four weeks.
Is XLA a primary immunodeficiency?
It is the first known immune deficiency, and is classified with other inherited (genetic) defects of the immune system, known as primary immunodeficiency disorders.
What is XLA in TensorFlow?
XLA (Accelerated Linear Algebra) is a domain-specific compiler for linear algebra that can accelerate TensorFlow models with potentially no source code changes.
How is XLA diagnosed?
The diagnosis of XLA can be confirmed by demonstrating the absence of BTK protein in monocytes or platelets or by the detection of a mutation in BTK in DNA. Almost every family has a different mutation in BTK; however, members of the same family usually have the same mutation.