PI occurs when a person’s immune system doesn’t work correctly or when parts of the system are missing. As a result, the immune system can’t make enough antibodies to fight infections effectively.


The exact prevalence of PI is unknown. In the United States, it is estimated that 1 in 2,000 children and 1 in 600 households are diagnosed with PI. Patients with PI often get one infection after another. These infections may never really improve as expected and may recur over time.

150,000 to 360,000


Source: Jiang F, Torgerson TR, Ayars AG. Allergy Asthma Clin Immunol. 2015.

150,000 to 360,000


Source: Jiang F, Torgerson TR, Ayars AG. Allergy Asthma Clin Immunol. 2015.


There’s no single cause. Many PI disorders are inherited, which means that the condition is passed down from one or both parents. Some immune system defects are caused by genetic factors in a person’s DNA. At one time, it was thought that PI was a disease of infants and small children. Now we know the symptoms of PI can appear later in life.

The following list shows 10 warning signs of PI.
You may have experienced one or more of them.

Icon for ear infections
Four or more new ear infections
within 1 year
Icon for sinus infections
Two or more serious sinus
infections within 1 year
Icon for antibiotics
Two or more months on
antibiotics with little effect
Icon for pneumonia
Two or more types of pneumonia
within one year
Icon for infant weight
Failure of an infant to gain weight
or grow normally
Icon for organ
Recurrent, deep skin or organ
Icon for fungal infection
Persistent thrush in mouth or
fungal infection on skin
Icon for intravenous antibiotics
Need for intravenous antibiotics
to clear infections
Icon for infections
Two or more deep-seated
infections, including septicemia
Icon for family
A family history of PI


There is no way to prevent primary immunodeficiency disorders because their causes are genetic. But you can take steps to help prevent infections that can occur due to a weakened immune system. Discuss the tips below with your doctor before giving them a try.

Icon for washing hands

Wash your hands regularly

Especially after being in public, and before eating

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Eat a healthy diet

Follow your doctor’s recommendation for a balanced diet

Icon for sleep schedule

Set a sleep schedule

Try to get the same number of hours of sleep every night

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Stay active

With your doctor’s approval, try light exercise – it may improve your overall health

Icon for oral hygiene

Practice good oral hygiene

Brush your teeth at least twice a day, and floss every night before bed

Icon for illness-causing germs

Limit exposure to illness-causing germs

Don’t give up your pandemic habits. Avoid people who are sick and practice social distancing

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Reduce stress levels

By exercising, meditating, or setting a sleep schedule. High stress levels can affect your immune system

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Ask your doctor about vaccines

Make sure you’re up to date on the vaccinations you need


One of the most common treatments for PI is immune globulin (Ig) replacement. Ig is a component of plasma. Plasma is the yellow-colored liquid left over when red blood cells are removed from whole blood that is donated by people with healthy immune systems. Ig is removed from the collected plasma and purified. It is then used to treat a variety of conditions, including PI.

Ig treatment can improve the health and quality of life for many patients with PI

Fewer respiratory tract (lung) infections | Less time spent in the hospital | Fewer missed work/school days
Group 131

Fewer respiratory tract (lung) infections

Group 130

Less time spent in the hospital

Group 129

Fewer missed work/school days

Important Safety Information for BIVIGAM®

BIVIGAM is an Immune Globulin Intravenous (Human), 10% Liquid, indicated for the treatment of primary humoral immunodeficiency (PI). This includes, but is not limited to, the humoral immune defect in common variable immunodeficiency (CVID), X linked agammaglobulinemia, congenital agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies (SCID).


  • Blood clots may happen with intravenous immune globulin products, including BIVIGAM. Before taking BIVIGAM, talk to your doctor if you:
    • Are of advanced age
    • Are unusually sedentary (long periods of sitting down or inactive)
    • Have a history of blood clots in the veins or arteries, cardiovascular (heart) problems or previous history of stroke
    • Are taking estrogen-containing medicines (birth control pills, hormone replacement therapy)
    • Have a permanent intravenous (IV) catheter
    • Have hyperviscosity (poor blood flow) of the blood (diseases such as multiple myeloma or other causes of elevated proteins in the blood)
  • Kidney problems, kidney failure, and death are more common in people who are taking human immune globulin products, especially those containing sucrose (sugar). BIVIGAM does not contain sucrose. Let your doctor know if you have kidney disease or diseases with kidney involvement, have a history of diabetes, or are older than 65 years of age. Your doctor should check your hydration level, blood infection risk, protein levels, and if you are taking any drugs that may damage your kidneys.
  • If you are at risk for blood clots or kidney problems, your doctor should adjust your starting dose of BIVIGAM and monitor your blood flow.

Tap for important safety information for BIVIGAM