What is PI?

Primary immunodeficiency diseases (PI) are a group of more than 400 rare, chronic disorders in which part of the body’s immune system is missing or functions improperly.1,2,3 Infections are caused by microorganisms such as bacteria, viruses, fungi and parasites. Nearly all of us get an infection once in a while - perhaps it's a minor cold, cough, or a cut that gets infected, or it may be something more serious like pneumonia. Normally, our immune system is a complicated and very efficient collaboration between cells and proteins that work together to defend us against infection.1 Most people recover quickly from an infection because the body's immune system defenses can usually eliminate the microorganisms that cause disease. But some of us are born with an immune system that is not able to effectively fight certain infections. PIs result when there is a defect in one or more of the components of the immune system.1

The impact of PI

There are estimated to be more than 400 different types of primary immunodeficiency diseases, affecting approximately 500,000 people in the US.2

Types of PI may differ depending on the specific defect in the immune system, but they all have one thing in common - patients have an increased susceptibility to infection. This can include frequent infections, infections that are difficult to treat, or infections with unusual organisms. In some forms of PI, organisms that cause only mild infections in healthy people may cause severe, life-threatening infections. These are called opportunistic infections.1

These infections can be located anywhere in the body, such as the ears, sinuses, lungs or intestinal tract.1 Besides being painful, frightening and frustrating, these constant infections can cause permanent damage to the ears or to the lungs.

 Although infections are the hallmark of PIs, they are not always the only health problem. Some PIs are associated with other immune disorders, such as anemia (reduced delivery of oxygen to tissues), arthritis, or autoimmune diseases. Other PIs involve more than the immune system; some, for instance, are associated with symptoms involving the heart, digestive tract, or nervous system. Some PIs retard growth and increase the risk of cancer.1

What can be done?

Today, physicians realize that PIs are sometimes relatively mild. Because of this, proper diagnosis might not occur until the teenage or adult years.1

Thanks to treatments available today, many PI diseases can be successfully managed, and some can even be cured. Many patients with PI are able to work, attend school, and participate fully in their communities.1

Treatment for PI usually involves "replacement therapy", in which the missing or defective antibodies are provided through regular infusions of intravenous immunoglobulins (IGIV). Replacement therapy can also be given under the skin, or subcutaneously for PI.

GAMMAKED is proven effective in patients with PI. In a clinical study, patients with PI who received Gammaked were protected from severe infections and acute sinusitis.4,5

Successfully controlling PI depends on prompt detection. Physicians, parents, and adult patients alike need to recognize when infections are more than "ordinary," so that treatment can be started in time to prevent permanent damage or life-threatening complications. Prompt diagnosis of PI has been improved over the years with advances in detection and confirmatory tests, as well as the implementation of newborn screening programs.

As soon as someone with primary immune deficiency has been identified in your family, you should check for similar symptoms in other family members so that they can be evaluated for PI as quickly as possible.

10 Warning Signs of Primary Immunodeficiency6

  1. Four or more new ear infections within 1 year
  2. Two or more serious sinus infections within 1 year
  3. Two or more months on antibiotics with little effect
  4. Two or more pneumonias within 1 year
  5. Failure of an infant to gain weight or grow normally
  6. Recurrent, deep skin or organ abscesses
  7. Persistent thrush in mouth or fungal infection on skin
  8. Need for intravenous antibiotics to clear infections
  9. Two or more serious infections including blood infections
  10. A family history of primary immunodeficiency

This information is not intended to be a substitute for professional medical care. You should consult your family physician or pediatrician for specific information on the diagnosis, treatment, and clinical care for patients with PI.

References: 1. IDF Patient and Family Handbook for Primary Immunodeficiency Diseases. 5th ed. Immune Deficiency Foundation. Towson, MD. 2013:2-151. http://primaryimmune.org/wp-content/uploads/2016/03/IDF-Patient-Family-Handbook-5th-Edition-2015-Reprint-Web.pdf. Accessed July 27, 2021. 2. National Institutes of Allergy and Infectious Diseases. Primary Immune Deficiency Diseases. https://www.niaid.nih.gov/diseases-conditions/primary-immune-deficiency-diseases-pidds. Accessed July 27, 2021. 3. About Primary Immunodeficiencies. Immune Deficiency Foundation Website. https://primaryimmune.org/about-primary-immunodeficiencies. Accessed July 27, 2021. 4. Roifman CM, Schroeder H, Berger M, et al. Comparison of the efficacy of IGIV-C, 10% (caprylate/chromatography) and IGIV-SD, 10% as replacement therapy in primary immune deficiency: a randomized double-blind trial. Int Immunopharmacol.2003;3:1325-33. 5. GAMMAKED [prescribing information]. Ft. Lee, NJ: Kedrion Biopharma; 2018. 6. Ten warning signs of primary immunodeficiency. The Jeffrey Modell Foundation Website. http://www.info4pi.org/library/educational-materials/10-warning-signs. Accessed July 27, 2021. 

Important Safety Information 


GAMMAKED™ [Immune Globulin Injection (Human) 10% Caprylate/Chromatography Purified] is an immune globulin injection that is used to treat primary humoral immunodeficiency (PI) in patients 2 years of age and older, idiopathic thrombocytopenic purpura (ITP) in adults and children, and chronic inflammatory demyelinating polyneuropathy (CIDP) in adults.

For treating PI: GAMMAKED can be given intravenously (IV, into a vein) or subcutaneously (SC, under the skin into the tissue) for PI. If you are infusing GAMMAKED subcutaneously for PI, DO NOT inject GAMMAKED into a blood vessel or directly into a muscle.

For treating CIDP or ITP: GAMMAKED should be given intravenously only (IV, into a vein) for CIDP or ITP. DO NOT infuse GAMMAKED subcutaneously (SC, under the skin into the tissue) for CIDP or ITP.


  1. Blood Clots (Thrombosis). Blood clots may occur in patients taking immune globulin intravenous (IGIV) products, including GAMMAKED. You may be at greater risk for blood clots if you are of advanced age, sit or lie for long periods, have a clotting condition or a history of blood clots, take estrogen hormones, have a central catheter, have thick blood, and/or if you have other conditions that put you at risk for cardiovascular disease. Blood clots may occur even if you do not have any of these known risk factors.
  2. Impaired kidney function or kidney failure. IGIV products, particularly those that contain sugar (sucrose), have been reported to be associated with kidney dysfunction and damage, kidney failure, and death. Kidney damage and kidney failure happen more often in patients receiving IGIV products containing sucrose. GAMMAKED does not contain sucrose. You may be at greater risk for kidney failure if you have kidney disease, diabetes, are over age 65, are seriously dehydrated, have a blood infection (sepsis), have a blood condition called paraproteinemia, or take drugs that can damage your kidneys.


  • Do not use GAMMAKED if: 
    • You have a history of severe allergic reactions to human immune globulin. Tell your healthcare provider if you have had a serious reaction to other medicines that contain human immune globulin. Ask if you are not sure.
    • You have an immunoglobulin A (IgA) deficiency and have antibodies to IgA and have a history of allergic reactions. Tell your healthcare provider if you have an IgA deficiency or ask if you are not sure.


  • Severe allergic reactions may occur with IGIV products, including GAMMAKED. IgA deficient patients who have antibodies against IgA are at greater risk of developing severe allergic reactions. Your healthcare provider should have medications, such as epinephrine, to immediately treat any sudden severe allergic reactions.
  • If you are receiving GAMMAKED, you could experience higher than normal levels of protein in your blood, thick blood, or low sodium (salt) in your blood. This may prevent your blood from flowing easily and possibly lead to blood clots.
  • Brain inflammation or brain swelling called Aseptic Meningitis Syndrome (AMS) may occur infrequently with IGIV products, including GAMMAKED, especially if you receive a high dose or a rapid infusion.
  • Blood damage called hemolysis and hemolytic anemia can develop after treatment with GAMMAKED. Your healthcare provider will closely monitor you for signs and symptoms of hemolysis and hemolytic anemia.
  • Swelling of the lungs may occur in patients following IGIV treatment, including GAMMAKED. Your healthcare provider will monitor you for signs of lung damage (also known as transfusion-related acute lung injury [TRALI]).
  • GAMMAKED is made from human blood and, therefore, carries a risk of transmitting infectious agents, such as viruses, the agent of the variant Creutzfeldt-Jakob disease (vCJD), or unknown infectious agents. You should consult with your healthcare provider if you have any questions or concerns.
  • Be sure to tell your healthcare provider about your recent history of vaccinations. Live vaccines for diseases like measles, mumps, rubella and varicella may not work as well for you while you are receiving GAMMAKED. Tell your healthcare provider that you are taking GAMMAKED before you receive any vaccination.


  • In clinical studies, the most common side effects of GAMMAKED were:       
    • Increased cough, stuffy nose, sore throat, headache, asthma, nausea, fever, diarrhea, and sinus infection, when administered intravenously to patients with PI.
    • Swelling and itching at the injection site, fatigue, headache, upper respiratory infection, joint pain, diarrhea, nausea, sinus infection, bronchitis, depression, allergic skin reactions, redness of the skin, migraine, muscle pain, viral infection, and fever, when administered subcutaneously to patients with PI.
    • Headache, bruising, vomiting, fever, nausea, rash, abdominal pain, back pain, and indigestion in patients with ITP.
    • Headache, fever, increased blood pressure, chills, rash, nausea, joint pain, and weakness in patients with CIDP.
  • During treatment with GAMMAKED, be sure to tell your healthcare provider about any unusual symptoms you experience as they may indicate a possible side effect. 

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/MedWatch, or call 1-800-FDA-1088. 

Please click here for the Full Prescribing Information, for more information including the Boxed Warning, a description of who should not take GAMMAKED, and dosing and administration information.

Gammaked is a registered trademark of Kedrion Biopharma Inc. 
©2021 Kedrion Biopharma Inc. All Rights Reserved. July 2021 GM-0110-05-2021