ABOUT PIDD
PIDD is a group of more than 400 hereditary, immunodeficiency disorders.1
The common feature of these is that the immune system is ineffective, thereby increasing the body's susceptibility to infections. People with PIDD could be at risk for chronic infections such as Epstein-Barr virus (EBV).2
Prevalence of PIDD
A 2007 survey by the Immune Deficiency Foundation estimated that there are 250,000 people in the United States, diagnosed with PIDD.3,4
According to the National Institutes of Health, about 500,000 people could be affected by PIDD in the country, suggesting that there could many who are yet to be diagnosed.2
A diagnosis of PIDD is often missed since healthy people also contract these infections from time to time. According to the IDF survey, it takes on an average about 12.4 years for patients to receive a diagnosis of PIDD from the time their symptoms begin.3
Signs and symptoms of PIDD
Patients with PIDD could present with some of the following symptoms.5
- Pneumonia
- Recurrent ear infections
- Recurrent sinus infections
- Failure to gain weight or grow normally
- Recurrent deep skin or organ abscesses
- Sepsis
- Persistent thrush in mouth
- Need for intravenous antibiotics to clear infections
Unique formulation
Manufactured to help protect a wide range of PIDD patients6,7*
Maximum potency
Proven efficacy to reduce the impact of PIDD on patients' lives8
Maximum purity
Proven tolerability profile8
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INDICATION
XEMBIFY® (immune globulin subcutaneous human–klhw) is a 20% immune globulin indicated for treatment of primary humoral immunodeficiency disease (PIDD) in patients 2 years of age and older. XEMBIFY is for subcutaneous administration only.
IMPORTANT SAFETY INFORMATION
WARNING: THROMBOSIS
- Thrombosis may occur with immune globulin products, including XEMBIFY. Risk factors may include: advanced age, prolonged immobilization, estrogens, indwelling vascular catheters, hyperviscosity, and cardiovascular risk factors. Thrombosis may occur in the absence of known risk factors
- For patients at risk of thrombosis, administer XEMBIFY at the minimum dose and infusion rate practicable. Ensure adequate hydration in patients before administration. Monitor for signs and symptoms of thrombosis and assess blood viscosity in patients at risk of hyperviscosity
Contraindications
XEMBIFY is contraindicated in patients who have had an anaphylactic or severe systemic reaction to the administration of human immune globulin. It is contraindicated in IgA-deficient patients with antibodies against IgA and a history of hypersensitivity.
Warnings and Precautions
Hypersensitivity. Severe hypersensitivity reactions may occur with immune globulin products, including XEMBIFY. In case of hypersensitivity, discontinue infusion immediately and institute appropriate treatment. XEMBIFY contains IgA. Patients with known antibodies to IgA may have a greater risk of developing potentially severe hypersensitivity and anaphylactic reactions.
Thrombosis. Thrombosis may occur following treatment with immune globulin products, including XEMBIFY. Thrombosis may occur in the absence of known risk factors. In patients at risk, administer at the minimum dose and infusion rate practicable. Ensure adequate hydration before administration. Monitor for signs and symptoms of thrombosis and assess blood viscosity in patients at risk of hyperviscosity.
Aseptic meningitis syndrome (AMS). AMS may occur with human immune globulin treatment, including XEMBIFY. Conduct a thorough neurological exam on patients exhibiting signs and symptoms to rule out other causes of meningitis. Discontinuation of treatment has resulted in remission within several days without sequelae.
Renal dysfunction/failure. Acute renal dysfunction/failure, acute tubular necrosis, proximal tubular nephropathy, osmotic nephrosis, and death may occur with use of human immune globulin products, especially those containing sucrose. XEMBIFY does not contain sucrose. Ensure patients are not volume-depleted prior to starting infusion. In patients at risk due to preexisting renal insufficiency or predisposition to acute renal failure, assess renal function prior to the initial infusion of XEMBIFY and again at appropriate intervals thereafter. If renal function deteriorates, consider discontinuation.
Hemolysis. XEMBIFY may contain blood group antibodies that may cause a positive direct antiglobulin reaction and hemolysis. Monitor patients for clinical signs and symptoms of hemolysis. If signs and symptoms are present after infusion, perform confirmatory lab testing.
Transfusion-related acute lung injury (TRALI). Noncardiogenic pulmonary edema may occur in patients following treatment with immune globulin products, including XEMBIFY. Monitor patients for pulmonary adverse reactions. If TRALI is suspected, perform appropriate tests for the presence of antineutrophil and anti-HLA antibodies in both the product and patient serum. TRALI may be managed using oxygen therapy with adequate ventilatory support.
Transmissible infectious agents. Because XEMBIFY is made from human blood, it may carry a risk of transmitting infectious agents, eg, viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent. No cases of transmission of viral diseases, vCJD, or CJD have ever been associated with the use of XEMBIFY.
Interference with lab tests. After infusion of XEMBIFY, passively transferred antibodies in the patient's blood may yield positive serological testing results, with the potential for misleading interpretation.
Adverse Reactions
The most common adverse reactions in ≥5% of subjects in the clinical trial were local adverse reactions, including infusion-site erythema (redness), infusion-site pain, infusion-site swelling (puffiness), infusion-site bruising, infusion-site nodule, infusion-site pruritus (itching), infusion-site induration (firmness), infusion-site scab, infusion-site edema, and systemic reactions including cough and diarrhea.
Drug Interactions
Passive transfer of antibodies may transiently interfere with the immune responses to live attenuated virus vaccines (eg, measles, mumps, rubella, and varicella).
Please see accompanying full Prescribing Information for XEMBIFY.
REFERENCES
- About primary immunodeficiencies. Immune Deficiency Foundation website. http://primaryimmune.org/about-primary-immunodeficiencies. Accessed May 14, 2020.
- Primary immune deficiency diseases (PIDDs). National Institute of Allergy and Infectious Diseases website. https://www.niaid.nih.gov/diseases-conditions/primary-immune-deficiency-diseases-pidds. Accessed May 14, 2020.
- Immune Deficiency Foundation. Primary Immunodeficiency Diseases in America: 2007. The Third National Survey of Patients. Prepared by Abt. SRBI, Inc. May 1, 2009.
- Boyle JM, Buckley RH. Population prevalence of diagnosed primary immunodeficiency diseases in the United States. J Clin Immunol. 2007;27(5):497-502.
- Jeffrey Modell Foundation Medical Advisory Board. 10 warning signs of primary immunodeficiency. Jeffrey Modell Foundation website. http://www.info4pi.org/library/educational-materials/10-warning-signs. Accessed January 11, 2021.
- Alonso W, Vandeberg P, Lang J, et al. Immune globulin subcutaneous, human 20% solution (Xembify®), a new high concentration immunoglobulin product for subcutaneous administration. Biologicals. 2020;64:34-40.
- Data on file, Grifols.
- Sleasman JW, Lumry WR, Hussain I, et al. Immune globulin subcutaneous, human - klhw 20% for primary humoral immunodeficiency: an open-label, Phase III study. Immunotherapy. 2019;11(16):1371-1386.
TERMS TO KNOW
SCIG, subcutaneous immune globulin.