Test ID: PPFE
Protoporphyrins, Fractionation, Whole Blood
Secondary ID 
A test code used for billing and in test definitions created prior to November 2011
NY State Approved Indicates the status of NY State approval and if the test is orderable for NY State clients.
Useful For Suggests clinical disorders or settings where the test may be helpful
Establishing a biochemical diagnosis of erythropoietic protoporphyria
Differential diagnosis of chronic intoxication from exposure to a variety of causes including heavy metals and chemicals
Differential diagnosis in some cases of iron-deficiency anemia
There are 2 test options: whole blood (PPFE/8739 Protoporphyrins, Fractionation, Whole Blood) and washed RBCs (PPFWE/31891 Protoporphyrins, Fractionation, Washed Erythrocytes). The whole blood option is easiest for clients but requires that the specimen arrive at Mayo Medical Laboratories within 72 hours of draw. When this cannot be ensured, washed RBCs should be submitted.
Genetics Test Information Provides information that may help with selection of the correct test or proper submission of the test request
Includes separate quantitation of zinc protoporphyrin and free protoporphyrin. Abstinence from alcohol is essential for at least 24 hours as alcohol suppresses enzyme activity, potentially leading to falsely increased results.
Testing Algorithm Delineates situation(s) when tests are added to the initial order. This includes reflex and additional tests.
The following algorithms are available in Special Instructions:
-Porphyria (Acute) Testing Algorithm
-Porphyria (Cutaneous) Testing Algorithm
Special Instructions and Forms Describes specimen collection and preparation information, test algorithms, and other information pertinent to test. Also includes pertinent information and consent forms to be used when requesting a particular test
Method Name A short description of the method used to perform the test
High-Performance Liquid Chromatography (HPLC) with Fluorescence Detection
Includes separate quantitation of zinc protoporphyrins and free protoporphyrins.
Reporting Name A shorter/abbreviated version of the Published Name for a test; an abbreviated test name
Aliases Lists additional common names for a test, as an aid in searching
Erythropoietic Protoporphyria
Free Protoporphyrin
Zinc Protoporphyrin
Specimen Type Describes the specimen type needed for testing
Specimen Required Defines the optimal specimen. This field describes the type of specimen required to perform the test and the preferred volume to complete testing. The volume allows automated processing, fastest throughput and, when indicated, repeat or reflex testing.
All porphyrin tests on whole blood can be performed on 1 draw tube.
Specimen must arrive within 72 hours of draw. If specimen cannot arrive within 72 hours, order PPFWE/31891 Protoporphyrins, Fractionation, Washed Erythrocytes.
Container/Tube:
Preferred: Green top (sodium heparin)
Acceptable: Metal free, lavender top (EDTA) or green top (lithium heparin)
Specimen Volume: Full tube
Collection Instructions:
1. Patient should abstain from alcohol for 24 hours.
2. Immediately place specimen on wet ice.
Additional Information: Include a list of medications the patient is currently taking.
Forms:
1. 1. New York Clients-Informed consent is required. Please document on the request form or electronic order that a copy is on file. An Informed Consent for Genetic Testing (Supply T576) is available in Special Instructions.
2. 2. If not ordering electronically, submit a Biochemical Genetics Request Form (Supply T439) with the specimen.
Specimen Minimum Volume Defines the amount of specimen required to perform an assay once, including instrument and container dead space. Submitting the minimum specimen volume makes it impossible to repeat the test or perform confirmatory or perform reflex testing. In some situations, a minimum specimen volume may result in a QNS (quantity not sufficient) result, requiring a second specimen to be collected.
Reject Due To Identifies specimen types and conditions that may cause the specimen to be rejected
| Hemolysis | Mild reject; Gross reject |
| Lipemia | NA |
| Icterus | NA |
| Other | NA |
Specimen Stability Information Provides a description of the temperatures required to transport a specimen to the laboratory. Alternate acceptable temperature(s) are also included.
| Specimen Type | Temperature | Time |
|---|---|---|
| Whole blood | Refrigerated | 72 hours |
Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
Zinc-complexed protoporphyrin can be increased in erythrocytes in association with:
-Iron deficiency anemia (the most common cause of elevated zinc protoporphyrin)
-Chronic intoxication by heavy metals (ie, lead) or various organic chemicals
-Hepatoerythropoietic porphyria
Noncomplexed (free) protoporphyrin is increased disproportionately in erythrocytes in cases of erythropoietic protoporphyria.
The following algorithms are available in Special Instructions:
-Porphyria (Acute) Testing Algorithm
-Porphyria (Cutaneous) Testing Algorithm
Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
FREE PROTOPORPHYRIN
<20 mcg/dL packed cells
ZINC-COMPLEXED PROTOPORPHYRIN
<60 mcg/dL packed cells
See The Heme Biosynthetic Pathway in Special Instructions.
Interpretation Provides information to assist in interpretation of the test results
Values of zinc-complexed protoporphyrin >60 mcg/dL suggest iron deficiency anemia or may indicate chronic intoxication by heavy metals or organic chemicals.
A predominance of noncomplexed (free) protoporphyrin indicates a diagnosis of erythropoietic protoporphyria.
Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Abstinence from alcohol is essential for at least 24 hours as alcohol suppresses enzyme activity, potentially leading to false-negative results.
It is essential to proceed expeditiously with obtaining, processing, and dispatching the specimen, precisely following the procedures specified in Specimen Required, paying special heed to maintaining low temperatures.
Clinical Reference Provides recommendations for further in-depth reading of a clinical nature
1. Ellefson RD: Porphyrinogens, porphyrins, and the porphyrias. Mayo Clin Proc 1982;57:454-458
2. Nuttall KL, Klee GG: Analytes of hemoglobin metabolism-porphyrins, iron, and bilirubin. In Tietz Fundamentals of Clinical Chemistry. Fifth edition. Edited by CA Burtis, ER Ashwood. Philadelphia, WB Saunders Book Company, 2001, pp 584-607
3. Anderson KE, Sassa S, Bishop DF, Desnick RJ: Disorders of heme biosynthesis: X-linked sideroblastic anemia and the porphyrias. In The Metabolic Basis of Inherited Disease. Eighth edition. Edited by CR Scriver, AL Beaudet, WS Sly, et al. New York, McGraw-Hill Medical Publishing Division, 2001, pp 2991-3062
4. Whatley SD, Ducamp S, Gouya L, et al: C-terminal in the ALAS2 gene lead to gain of function and cause X-linked dominant protoporphyria without anemia or iron overload. Am J Hum Genet 2008 Sep;83(3):408-414
Method Description Describes how the test is performed and provides a method-specific reference
Extraction followed by fractionation by HPLC. Zinc protoporphyrins and free protoporphyrins are separately quantitated.(Ford RE, Ellefson RD: Quantitation of erythrocyte protoporphyrins and zinc protoporphyrin by high-pressure liquid chromatography. Clin Chem 1984;30:972)
Day(s) and Time(s) Test Performed Outlines the days and times the test is performed. This field reflects the day and time the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time required before the test is performed. Some tests are listed as continuously performed, which means assays are performed several times during the day.
Monday through Friday, 8 a.m.
Analytic Time Defines the amount of time it takes the laboratory to setup and perform the test. This is defined in number of days. The shortest interval of time expressed is "same day/1 day," which means the results may be available the same day that the sample is received in the testing laboratory. One day means results are available 1 day after the sample is received in the laboratory.
Maximum Laboratory Time Defines the maximum time from specimen receipt at Mayo Medical Laboratories until the release of the test result
Specimen Retention Time Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
Performing Laboratory Location The location of the laboratory that performs the test
Test Classification Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer's instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR), Investigation Use Only (IUO) product, or a Research Use Only (RUO) product.
CPT Code Information Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Medical Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
84202
LOINC® Code Information Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the result codes returned for this test or profile.
| Result ID | Reporting Name | LOINC Code |
|---|---|---|
| 2327 | Zinc-Complexed Protoporphyrin | 33007-6 |
| 2326 | Free Protoporphyrin | 2892-8 |
| 29511 | Interpretation | 59462-2 |
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