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HelpTest Code UPGDW Uroporphyrinogen Decarboxylase, Washed Erythrocytes
Reporting Name
UPG Decarboxylase, RBCPerforming Laboratory
Mayo Clinic Laboratories in Rochester
Useful For
Diagnosis of porphyria cutanea tarda type II and hepatoerythropoietic porphyria
Method Name
High-Performance Liquid Chromatography (HPLC)/Incubation of Lysed Erythrocytes
Ordering Guidance
Porphyria cutanea tarda (PCT) type I (sporadic), the most common form of PCT, exhibits normal erythrocyte enzyme activity. The preferred test for diagnosis is PQNU / Porphyrins, Quantitative, 24 Hour, Urine or PQNRU / Porphyrins, Quantitative, Random, Urine.
Necessary Information
Include a list of medications the patient is currently taking.
Specimen Required
Patient Preparation: Patient must not consume any alcohol for 24 hours before specimen collection. This is essential as alcohol suppresses enzyme activity for 24 hours after ingestion.
Collection Container/Tube:
Preferred: Green top (sodium heparin)
Acceptable: Lavender top (EDTA) or green top (lithium heparin)
Submission Container/Tube: Plastic vial
Specimen Volume: Entire washed erythrocyte suspension
Collection Instructions: Collect and process whole blood specimen as follows:
1. Transfer entire specimen to a 12-mL graduated centrifuge tube.
2. Centrifuge specimen at 4° C for 10 minutes at 2000 rpm.
3. Record volume of packed cells and the total volume of the specimen.
4. Discard supernatant plasma.
5. Wash erythrocytes 2 times by resuspension with 5 mL of cold 0.9% saline, discarding supernatant after each washing.
6. Resuspend packed cells to the original total volume with 0.9% saline. Invert specimen gently to mix.
Specimen Type
Washed RBCSpecimen Minimum Volume
1 mL of washed and resuspended erythrocytes
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Washed RBC | Frozen (preferred) | 14 days | |
| Refrigerated | 14 days | ||
| Ambient | 4 days |
Reject Due To
| All specimens will be evaluated at Mayo Clinic Laboratories for test suitability. |
Reference Values
≥1.00 RU (normal)
0.80-0.99 RU (indeterminate)
<0.80 RU (indicative of PCT type II)
RU = Relative Units
Interpretation
Abnormal results are reported with a detailed interpretation that may include an overview of the results and their significance, a correlation to available clinical information provided with the specimen, differential diagnosis, recommendations for additional testing when indicated, and available, and a phone number to reach one of the laboratory directors in case the referring physician has additional questions.
Day(s) Performed
Tuesday, Thursday
Report Available
2 to 6 daysTest Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
82657
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| UPGDW | UPG Decarboxylase, RBC | 49596-0 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 31892 | UPG Decarboxylase, RBC | 49596-0 |
| 606381 | Interpretation | 59462-2 |
| 606382 | Reviewed By | 18771-6 |
Forms
1. New York Clients-Informed consent is required. Document on the request form or electronic order that a copy is on file. The following documents are available:
-Informed Consent for Genetic Testing (T576)
-Informed Consent for Genetic Testing-Spanish (T826)
2. If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.
Testing Algorithm
The workup of patients with a suspected porphyria is most effective when following a stepwise approach. See Porphyria (Cutaneous) Testing Algorithm or call 800-533-1710 to discuss testing strategies. If guidance is needed for an acute form of porphyria, the Porphyria (Acute) Testing Algorithm is also available.