For US Healthcare Professionals
Benefits of dihydrorhodamine (DHR) testing
The dihydrorhodamine (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 detect gp91phox carriers
- High sensitivity and ability to quantitatively assess residual superoxide production
Speak with a clinical science associate (CSA) to learn more about the DHR Test.
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*The DHR test is also referred to as the neutrophil oxidative burst (NOXB1) assay for assessing neutrophil superoxide4
Example histograms showing 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.
Learn more about testing for CGD
Example histograms showing 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.
Individual without CGD
The neutrophils respond to the stimulus, meaning the cells are functioning.
Patient with X-linked CGD
The cells do not respond to the stimulus, meaning they also don’t respond to specific bacteria and fungi.
DHR @ FITC-ADHR @ FITC-A
| VARIABLE | VALUE (PATIENT) | UNIT | NORMAL CUTOFF | VALUE (UNRELATED HEALTHY DONOR) |
|---|---|---|---|---|
ANC | 4235 | Cell/μL | 1700-7000† | 5987 |
% PMA ox-DHR+ | 4.1 | % | ≥95% | 100.0 |
MFI PMA ox-DHR+ | 6.73 | MFI | ≥60 | 78.25 |
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.
X-linked female CGD carrier§
A carrier has 2 populations of the cells—cells that respond to the stimulus and cells that don’t.
§Usually a female with a healthy and mutated allele for gp91phox.
Patient with autosomal recessive CGD
A reduced response to the stimulus is demonstrated. Both males and females can present with autosomal recessive CGD.
| VARIABLE | VALUE (PATIENT) | UNIT | NORMAL CUTOFF | VALUE (UNRELATED HEALTHY DONOR) |
|---|---|---|---|---|
ANC | 2743 | Cell/μL | 1700-7000† | 5987 |
% PMA ox-DHR+ | 53.8 | % | ≥95% | 100.0 |
MFI PMA ox-DHR+ | 31.34 | MFI | ≥60 | 78.25 |
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 al5 Leiding et al (2013).6
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.
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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. 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=354405. 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. 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
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