Testing for chronic granulomatous disease (CGD)

Benefits of dihydrorhodamine (DHR) testing*

The DHR test has the ability to assess neutrophil superoxide production, a risk indicator for serious infection. Resulting in fewer false-negative test results than the NBT test, the DHR test is known for its1-3

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

*The DHR test is also referred to as the neutrophil oxidative burst (NOXB1) assay for assessing neutrophil superoxide.4

The example histograms below show neutrophil NADPH oxidase activity in both an unstimulated sample and a sample stimulated with phorbol myristate (PMA). These values are representations of possible DHR outcomes. Because of heterogeneity in disease severity and genotype, outcomes will vary.

Adapted from Jirapongsananuruk O, et al (2003)5 and Leiding JW, et al (2013).6

†PMA is an activator used to stimulate neutrophil NADPH oxidase activity.

‡Usually a female with a healthy and mutated allele for gp91phox.

MFI, mean fluorescence intensity; NADPH, nicotinamide adenine dinucleotide phosphate; ox, oxidative.

Stay informed about ACTIMMUNE and CGD
Sign Up for Updates

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; 1993-2022. 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. 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. 4. Test ID: NOXB. Mayo Medical Laboratories website. Published October 8, 2014. Accessed March 4, 2014. http://www.mayomedicallaboratories.com/test-updates/attachment.php?id=35440 5. 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. 6. Leiding JW, Malech HL, Holland SM. Chronic granulomatous disease. Clinical Focus on Primary Immunodeficiencies: Chronic Granulomatous Disease. Immune Deficiency Foundation; 2013.

Approved Uses and 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-1b, 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
    • Myelosuppression or receiving other potentially myelosuppressive agents
    • Severe renal insufficiency
    • Age <1 year
  • Monitoring:
    • Before starting ACTIMMUNE and every 3 months during treatment, hematologic tests, blood chemistries, and urinalysis are recommended for all patients
    • Patients begun on ACTIMMUNE before the age of 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

USE IN SPECIFIC POPULATIONS
  • ACTIMMUNE should be used during pregnancy only if the potential benefit to the patient outweighs the potential risk to the fetus
  • It is not known if ACTIMMUNE is excreted in human milk, so either ACTIMMUNE or nursing should be discontinued dependent on the importance of the drug to the patient
  • In younger patients, long-term effects of ACTIMMUNE on fertility are not known
  • In animal studies, both male and female fertility was negatively impacted by doses significantly higher than the maximum clinical dose

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

Please see Full Prescribing Information for additional safety information.

Approved Uses and Important Safety Information

Approved Uses and Important Safety Information

INDICATIONS AND USAGE

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

Approved Uses and Important Safety Information

Approved Uses and Important Safety Information

INDICATIONS AND USAGE

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

Approved Uses and 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-1b, 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
    • Myelosuppression or receiving other potentially myelosuppressive agents
    • Severe renal insufficiency
    • Age <1 year
  • Monitoring:
    • Before starting ACTIMMUNE and every 3 months during treatment, hematologic tests, blood chemistries, and urinalysis are recommended for all patients
    • Patients begun on ACTIMMUNE before the age of 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

USE IN SPECIFIC POPULATIONS
  • ACTIMMUNE should be used during pregnancy only if the potential benefit to the patient outweighs the potential risk to the fetus
  • It is not known if ACTIMMUNE is excreted in human milk, so either ACTIMMUNE or nursing should be discontinued dependent on the importance of the drug to the patient
  • In younger patients, long-term effects of ACTIMMUNE on fertility are not known
  • In animal studies, both male and female fertility was negatively impacted by doses significantly higher than the maximum clinical dose

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

Please see Full Prescribing Information for additional safety information.
arrow_upward