Health Library Logo

Health Library

X Linked Agammaglobulinemia

Overview

X-linked agammaglobulinemia, often called XLA, is a genetic condition that affects the immune system. This means it's passed down through families. Because the immune system is weakened, people with XLA have a harder time fighting off infections. Common infections include those of the inner ear, sinuses, lungs (respiratory system), the blood, and internal organs. Importantly, XLA almost always affects boys. While girls can carry the genes that cause the condition, they usually don't get sick from it. Most people with XLA are diagnosed when they're babies or young children, after experiencing several infections. However, some people aren't diagnosed until they're older, even into adulthood.

Symptoms

Many babies with X-linked agammaglobulinemia (XLA) seem normal for the first few months of life. This is because they have antibodies, a type of protein that fights infection, passed on from their mothers before birth. However, as these maternal antibodies disappear from the baby's system, they become much more vulnerable to bacterial infections. These infections can be serious and even life-threatening. They can affect various parts of the body, including the ears, lungs, sinuses, and skin. Babies with XLA, especially boys, often have noticeable differences in their immune system development. Specifically, they tend to have very small tonsils and may have small or no lymph nodes. These lymph nodes are crucial parts of the body's infection-fighting system.

Causes

X-linked agammaglobulinemia is a genetic condition. It happens when a person's DNA, specifically a certain gene, has a problem. This problem prevents their body from making enough antibodies. Antibodies are proteins that your body uses to fight off germs and infections. Without enough antibodies, people with this condition are more vulnerable to infections. In about 4 out of every 10 cases (40%), someone in the person's family also has the condition, suggesting a hereditary link.

Complications

People with XLA can generally live normal lives, engaging in activities appropriate for their age. However, because XLA can lead to repeated infections, these infections need close monitoring and treatment. Repeated infections can harm organs and be serious, even life-threatening. Potential problems include:

  • Chronic lung disease: This means long-term lung problems that can last a lifetime.
  • Higher chance of some cancers: People with XLA may have a greater risk of developing certain types of cancer compared to those without the condition.
  • Infectious arthritis: This is joint pain and inflammation caused by infection.
  • Increased risk of infections in the brain and spinal cord from live vaccines: Live vaccines can sometimes cause infections in the central nervous system (brain and spinal cord) in people with XLA. This is why careful consideration of vaccine choices is important.

Address: 506/507, 1st Main Rd, Murugeshpalya, K R Garden, Bengaluru, Karnataka 560075

Disclaimer: August is a health information platform and its responses don't constitute medical advise. Always consult with a licenced medical professional near you before making any changes.

Made in India, for the world