Agammaglobulinemia

Overview

Agammaglobulinemia is a rare, usually inherited immune disorder in which the body makes very few B cells and little or no antibodies. The most common form, X-linked agammaglobulinemia, mainly affects boys and often becomes noticeable in early childhood, once the antibodies passed on from the mother fade. Without antibodies, bacterial and some viral infections can be frequent and serious.

Common Signs and Symptoms

• Repeated ear, sinus or lung infections
• Pneumonia or bronchitis that keeps coming back
• Infections of the skin, gut or joints
• Infections that respond poorly to usual antibiotics
• Small or absent tonsils and lymph nodes

When to Contact a Provider

Contact a provider about frequent or severe infections, infections that needed IV antibiotics, or a family history of agammaglobulinemia. If you are already on treatment, report a fever or new infection promptly.

How Treatment May Help

The main treatment is lifelong immunoglobulin (antibody) replacement, prescribed by an immunologist. Regular doses supply antibodies the body cannot make and may help reduce how often infections happen and how severe they are. Immunoglobulin can be given by IV infusion (IVIG) every few weeks or as more frequent injections under the skin (SCIG).

Therapies That May Be Available at Infuse Care

• IVIG (immune globulin intravenous): IV infusion, often every three to four weeks; dose and schedule are set by your immunologist

Safety and Monitoring

Headache, chills, tiredness and flushing can occur during or after an infusion. Rare but serious risks include blood clots and kidney problems, so your provider reviews your health history and our nurses adjust the infusion rate and encourage hydration. Live vaccines are generally avoided.

Your prescribing provider decides which treatment is right for you. Infuse Care administers prescribed therapies after a referral, a clinical review and any required insurance authorization, and we keep your provider's office updated on your care.