Hexosaminidase B (Beta) Activity in Whole Blood
This test measures the activity of the enzyme hexosaminidase B, which plays a role in breaking down certain sugars in your blood. It may be used to diagnose or monitor conditions affecting lysosomal storage disorders.
["This test helps identify genetic enzyme deficiencies linked to rare metabolic diseases."]
Key facts at a glance
- LOINC
- 24073-9
- Short code
- Hexosaminidase B WBC-cCnt
- Synonyms
- Hexosaminidase B WBC-cCnt
- Category
- chemistry
- Sample
- serum
- Container
- Red-top tube (SST)
- Units
- N/A
- Ref low
- 4.5
- Ref high
- 11
- Critical range
- 2 N/A – 30 N/A
- Fasting
- false
- Population
- adult
- Sex
- all
- Age range
- 18–65 years
- CPT
- 85004
- SNOMED
- 900000000000003002
- Specimen volume
- 5 mL
- Stability
- Room temperature: 4h, Refrigerated: 24h, Frozen: 7d
- Hemolysis max
- 500
- Lipemia max
- 1000
- Icterus max
- 500
- TAT
- 4–24 hours
- Order
- routine
Why This Test Might Be Ordered
Your healthcare provider may recommend this test if you have symptoms like developmental delays, muscle weakness, or organ enlargement. It’s also used in genetic testing for conditions like Sanfilippo syndrome, a group of lysosomal storage disorders.
What to Expect Before the Test
No special preparation is needed. You’ll provide a small sample of whole blood, typically drawn from your arm. The test is usually done in a lab setting and results may take several days to weeks to process.
How the Test Works
The blood sample is analyzed to measure the enzyme activity of hexosaminidase B. This involves breaking down a specific sugar substrate and measuring how quickly the enzyme converts it, which reflects its functional level in your blood.
Possible Results and Interpretation
Results may be compared to a reference range for healthy individuals, though exact values can vary by lab. Low levels of hexosaminidase B may indicate a genetic disorder affecting lysosomal function. Your doctor will discuss results in the context of your symptoms and medical history.
Reference range
Adult: 4.5-11.0 x 10^9/LN/A
No special preparation required. Stay well hydrated.
Clinical interpretation
How abnormal results are interpreted and what conditions they suggest.
Clinical Significance
Short name
Hex B Activity
Frequently asked questions
What does a low or high hexosaminidase B result mean?
Can diet or lifestyle changes affect this test?
How often will I need this test?
Sources
- 1
- 2Lab Tests OnlineSource
- 3
Click any link to access the full source. PubMed Central (PMC) links provide free full-text access.
This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 16, 2026. · Read our editorial policy