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Common Questions

What is the safety profile of ACTIMMUNE®?

What is the efficacy of ACTIMMUNE®?

What is the preferred test for CGD?

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Benefits of dihydrorhodamine (DHR) testing and how to order the test

Historically, the nitroblue tetrazolium (NBT) test has been the recognized diagnostic test for chronic granulomatous disease (CGD). Relying on light microscopy, NBT provides only a qualitative determination of phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase activity. The NBT test is also open to operator subjectivity and can produce false-negative results.1-3

The dihydrorhodamine (DHR) test* is the preferred method for diagnosing CGD. Resulting in fewer false-negative test results than the NBT test, the DHR test is known for its1-4:

  • Relative ease of use
  • Objectivity without requiring significant operator experience
  • Ability to distinguish between X-linked and autosomal recessive forms of CGD
  • Ability to detect gp91phox carriers
  • High sensitivity and ability to quantitatively assess residual superoxide production

Speak with a clinical science associate (CSA) about testing for CGD with the DHR test.

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*The DHR test is also referred to as the neutrophil oxidative burst (NOXB1) assay for assessing neutrophil superoxide production.5

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Examples of pre- and postactivation DHR histograms

Pre- and post-activation DHR histograms

Pre- and post-activation DHR histograms

X-linked CGD Patient

X-linked chronic granulomatous disease (CGD) DHR test results graph

X-linked chronic granulomatous disease (CGD) DHR test results graph

X-linked chronic granulomatous disease (CGD) DHR test results chart

X-linked chronic granulomatous disease (CGD) DHR test results chart

Abbreviations: ANC, absolute neutrophil count; FITC, fluorescein isothiocyanate; MFI, mean fluorescence intensity; PMA, phorbol myristate acetate.

Results:

Result is consistent with associated X-linked CGD in a male patient.

Autosomal recessive CGD Patient

Autosomal recessive chronic granulomatous disease (CGD) DHR test results graph

Autosomal recessive chronic granulomatous disease (CGD) DHR test results graph

Autosomal recessive chronic granulomatous disease (CGD) DHR test results char

Autosomal recessive chronic granulomatous disease (CGD) DHR test results chart

Abbreviations: ANC, absolute neutrophil count; FITC, fluorescein isothiocyanate; MFI, mean fluorescence intensity; PMA, phorbol myristate acetate.

aPMA is an activator used to stimulate neutrophil NADPH oxidase activity. bUsually a female with a healthy and a mutated allele for gp91phox. Adapted from Jirapongsananuruk et al6 Leiding et al (2013)7.

Results:

The result is consistent with associated autosomal recessive CGD in a female patient.

†The appropriate age-related reference values for absolute neutrophil count will be provided on the report.

These values are representations of possible DHR outcomes. Because of heterogeneity in disease severity and genotype, outcomes will vary.

Learn more about ACTIMMUNE® or the DHR test

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References: 1. Leiding JW, Holland SM. Chronic granulomatous disease. In: Pagon RA, Adam MP, Ardinger HH, et al, eds. GeneReviews. Seattle, WA: University of Washington, Seattle; 1993-2016. 2. Thomsen IP, Smith MA, Holland SM, Creech CB. A comprehensive approach to the management of children and adults with chronic granulomatous disease. J Allergy Clin Immunol Pract. 2016;4(6):1082-1088. 3. Wolfe LC, Keefe E. Pediatric chronic granulomatous disease clinical presentation. Medscape website. http://emedicine.medscape.com/article/956936-clinical. Updated November 17, 2014. Accessed April 29, 2015. 4. Kuhns DB, Alvord WG, Heller T, et al. Residual NADPH oxidase and survival in chronic granulomatous disease. N Engl J Med. 2010;363(27):2600-2610. 5. Test ID: NOXB. Mayo Medical Laboratories website. http://www.mayomedicallaboratories.com/test-updates/attachment.php?id=35440. Published October 8, 2014. Accessed March 4, 2014. 6. Jirapongsananuruk O, Malech HL, Kuhns DB, et al. Diagnostic paradigm for evaluation of male patients with chronic granulomatous disease, based on the dihydrorhodamine 123 assay. J Allergy Clin Immunol. 2003;111(2):374-379. 7. Leiding JW, Malech HL, Holland SM. Chronic granulomatous disease. Clinical Focus on Primary Immunodeficiencies. 2013;15:1-9.

Important Safety Information

INDICATIONS AND USAGE

ACTIMMUNE® (Interferon gamma-1b) is indicated:

  • For reducing the frequency and severity of serious infections associated with Chronic Granulomatous Disease
  • For delaying time to disease progression in patients with severe, malignant osteopetrosis

IMPORTANT SAFETY INFORMATION

CONTRAINDICATIONS

  • In patients who develop or have known hypersensitivity to interferon-gamma, E coli-derived products, or any component of the product

WARNINGS AND PRECAUTIONS

  • ACTIMMUNE should be used with caution in patients with:
    • Pre-existing cardiac conditions, including ischemia, congestive heart failure, or arrhythmia
    • Seizure disorders or compromised central nervous system function; reduce dose or discontinue
    • Myelosuppression, or receiving other potentially myelosuppressive agents; consider dose reduction or discontinuation of therapy
    • Severe renal insufficiency
    • Age <1 year
  • Monitoring:
    • Patients begun on ACTIMMUNE before age 1 year should receive monthly assessments of liver function. If severe hepatic enzyme elevations develop, ACTIMMUNE dosage should be modified
    • Monitor renal function regularly when administering ACTIMMUNE in patients with severe renal insufficiency; accumulation of interferon gamma-1b may occur with repeated administration. Renal toxicity has been reported in patients receiving ACTIMMUNE
  • Pregnancy, Lactation, and Fertility:
    • ACTIMMUNE should be used during pregnancy only if the potential benefit outweighs the potential risk to the fetus
    • Use of ACTIMMUNE by lactating mothers is not recommended. ACTIMMUNE or nursing should be discontinued dependent on the importance of the drug to the mother
    • Long-term effects of ACTIMMUNE on fertility are not known

DRUG INTERACTIONS

  • Concomitant use of drugs with neurotoxic, hematotoxic, or cardiotoxic effects may increase the toxicity of interferons
  • Avoid simultaneous administration of ACTIMMUNE with other heterologous serum protein or immunological preparations (eg, vaccines)

ADVERSE REACTIONS

  • The most common adverse experiences occurring with ACTIMMUNE therapy are “flu-like” symptoms such as fever, headache, chills, myalgia, or fatigue, which may decrease in severity as treatment continues, and may be minimized by bedtime administration of ACTIMMUNE. Acetaminophen may be used to prevent or partially alleviate the fever and headache
  • Isolated cases of acute serious hypersensitivity reactions have been observed in patients receiving ACTIMMUNE
  • Reversible neutropenia, thrombocytopenia, and elevations of AST and/or ALT have been observed during ACTIMMUNE therapy
  • At doses 10 times greater than the weekly recommended dose, ACTIMMUNE may exacerbate pre-existing cardiac conditions, or may cause reversible neurological effects such as decreased mental status, gait disturbance, and dizziness

Important Safety Information

INDICATIONS AND USAGE

ACTIMMUNE® (Interferon gamma-1b) is indicated:

  • For reducing the frequency and severity of serious infections associated with Chronic Granulomatous Disease
  • For delaying time to disease progression in patients with severe, malignant osteopetrosis
IMPORTANT SAFETY INFORMATION

CONTRAINDICATIONS

  • In patients who develop or have known hypersensitivity to interferon-gamma, E coli-derived products, or any component of the product

WARNINGS AND PRECAUTIONS

  • ACTIMMUNE should be used with caution in patients with:
    • Pre-existing cardiac conditions, including ischemia, congestive heart failure, or arrhythmia
    • Seizure disorders or compromised central nervous system function; reduce dose or discontinue
    • Myelosuppression, or receiving other potentially myelosuppressive agents; consider dose reduction or discontinuation of therapy
    • Severe renal insufficiency
    • Age <1 year
  • Monitoring:
    • Patients begun on ACTIMMUNE before age 1 year should receive monthly assessments of liver function. If severe hepatic enzyme elevations develop, ACTIMMUNE dosage should be modified
    • Monitor renal function regularly when administering ACTIMMUNE in patients with severe renal insufficiency; accumulation of interferon gamma-1b may occur with repeated administration. Renal toxicity has been reported in patients receiving ACTIMMUNE
  • Pregnancy, Lactation, and Fertility:
    • ACTIMMUNE should be used during pregnancy only if the potential benefit outweighs the potential risk to the fetus
    • Use of ACTIMMUNE by lactating mothers is not recommended. ACTIMMUNE or nursing should be discontinued dependent on the importance of the drug to the mother
    • Long-term effects of ACTIMMUNE on fertility are not known

DRUG INTERACTIONS

  • Concomitant use of drugs with neurotoxic, hematotoxic, or cardiotoxic effects may increase the toxicity of interferons
  • Avoid simultaneous administration of ACTIMMUNE with other heterologous serum protein or immunological preparations (eg, vaccines)

ADVERSE REACTIONS

  • The most common adverse experiences occurring with ACTIMMUNE therapy are “flu-like” symptoms such as fever, headache, chills, myalgia, or fatigue, which may decrease in severity as treatment continues, and may be minimized by bedtime administration of ACTIMMUNE. Acetaminophen may be used to prevent or partially alleviate the fever and headache
  • Isolated cases of acute serious hypersensitivity reactions have been observed in patients receiving ACTIMMUNE
  • Reversible neutropenia, thrombocytopenia, and elevations of AST and/or ALT have been observed during ACTIMMUNE therapy
  • At doses 10 times greater than the weekly recommended dose, ACTIMMUNE may exacerbate pre-existing cardiac conditions, or may cause reversible neurological effects such as decreased mental status, gait disturbance, and dizziness