Overview
X-linked agammaglobulinemia (pronounced “ay-ga-muh-glaa-byou-luh-NEE-mee-uh”), or XLA, is a genetic condition where your body doesn’t make enough mature B-cells. B-cells are an important part of your immune system. They make proteins (antibodies) that help you fight off illnesses. People with XLA aren’t able to fight off illnesses easily and get sick often.
Because B-cells are important for developing certain immune system tissues, people with XLA often have underdeveloped or absent lymph nodes, tonsils and adenoids. Because of how you inherit it, it almost always affects people assigned male at birth (AMAB).
XLA is also called:
- Bruton’s agammaglobulinemia.
- Congenital agammaglobulinemia.
- Hypogammaglobulinemia.
Hypogammaglobulinemia is also a name used for common variable immunodeficiency (CVID), a condition similar to XLA. CVID is usually less severe and typically not diagnosed until adulthood. Providers typically diagnose people with XLA before the age of 1 or as young children.
What’s the difference between X-linked agammaglobulinemia and severe combined immunodeficiency (SCID)?
The difference between X-linked agammaglobulinemia and severe combined immunodeficiency (SCID) is that XLA affects your B-cells, and SCID affects your T-cells. Both are genetic conditions that affect your immune system and make you get sick frequently.
How common is X-linked agammaglobulinemia?
X-linked agammaglobulinemia is rare. It’s more common in people AMAB. Approximately 1 in 200,000 people AMAB are born with XLA.
Symptoms
When to see a doctor
Complications
- High blood pressure.
- Diabetes.
- Heart failure.
- Some types of heart valve disease.
Prevention
- Control high blood pressure, high cholesterol and diabetes.
- Don't smoke or use tobacco.
- Eat a diet that's low in salt and saturated fat.
- Exercise at least 30 minutes a day on most days of the week unless your health care team says not to.
- Get good sleep. Adults should aim for 7 to 9 hours daily.
- Maintain a healthy weight.
- Reduce and manage stress.
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