Porphyrins are a group of chemicals that help form many important substances in the body, including hemoglobin, the protein in red blood cells that carries oxygen in the blood.

This article discusses the test to measure the total amount of porphyrins in your blood.

See also: Porphyrins - urine

Protoporphyrin levels; Porphyrins - total; Coproporphyrin levels; PROTO test

Blood is typically drawn from a vein, usually from the inside of the elbow or the back of the hand. The site is cleaned with germ-killing medicine (antiseptic). The health care provider wraps an elastic band around the upper arm to apply pressure to the area and make the vein swell with blood.

Next, the health care provider gently inserts a needle into the vein. The blood collects into an airtight vial or tube attached to the needle. The elastic band is removed from your arm.

Once the blood has been collected, the needle is removed, and the puncture site is covered to stop any bleeding.

In infants or young children, a sharp tool called a lancet may be used to puncture the skin and make it bleed. The blood collects into a small glass tube called a pipette, or onto a slide or test strip. A bandage may be placed over the area if there is any bleeding.

The sample is then placed in ice and taken immediately to the laboratory. Three porphyrins can normally be measured in small amounts in human blood. They are:

• Coproporphyrin
• Protoporphyrin (PROTO)
• Uroporphyrin

Protoporphyrin is normally found in the highest amount. Additional tests are needed to show the levels of specific porphyrins.

You should not eat for 12 - 14 hours before this test. You may drink water right before the test. Failure to follow these restrictions may affect the test results.

When the needle is inserted to draw blood, you may feel moderate pain, or only a prick or stinging sensation. Afterward, there may be some throbbing.

This test is used to diagnose porphyrias, a group of rare disorders often passed down through family members.

It may also be used along with other tests to diagnose lead poisoning and certain nervous system and skin disorders.

This test specifically measures total porphyrin levels, but reference values (a range of values seen in a group of healthy people) for the individual components are also included:

• Total porphyrin levels: 16 to 60 mcg/dL
• Coproporphyrin levels: < 2 mcg/dL
• Protoporphyrin levels: 16 to 60 mcg/dL
• Uroporphyrin levels: < 2 mcg/dL

Note: mcg/dL = micrograms per deciliter

Normal value ranges may vary slightly among different laboratories. Talk to your doctor about the meaning of your specific test results.

Increased levels of coproporphyrins may indicate:

• Congenital erythropoietic porphyria
• Hepatic coproporphyria
• Sideroblastic anemia

Increased protoporphyrin levels may indicate:

• Anemia of chronic disease
• Erythropoietic protoporphyria
• Increased erythropoiesis
• Infection
• Iron deficiency anemia
• Lead poisoning
• Sideroblastic anemia
• Thalassemia
• Variegate porphyria

Increased uroporphyrin levels may indicate:

• Congenital erythropoietic porphyria

Veins and arteries vary in size from one patient to another and from one side of the body to the other. Obtaining a blood sample from some people may be more difficult than from others.

Other risks associated with having blood drawn are slight but may include:

• Excessive bleeding
• Fainting or feeling light-headed
• Hematoma (blood accumulating under the skin)
• Infection (a slight risk any time the skin is broken)

Anderson KE. The porphyrias. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 229.

Porphyrins help form many important substances in the body including hemoglobin, the protein in red blood cells that carries oxygen in the blood.

Porphyrins can be found in urine. A urine porphyrins test measures the amount of porphyrins in the urine.

Uroporphyrin

A 24-hour urine sample is needed.

• On day 1, urinate into the toilet when waking up in the morning.
• For the next 24 hours, every time you urinate, collect the urine in a special container. Keep it in the refrigerator or a cool place during the collection period.
• On day 2, urinate into the container in the morning when waking up. Close the container, and label it with your name, date, and time you finished.
• Return the container to your health care provider as instructed.

In infants:

Thoroughly wash the area around the urethra (the area where urine exits). Open the urine collection bag provided by your doctor. For boys, place the entire penis in the bag and attach it to the surrounding skin. For girls, place the bag over the labia.

You can diaper the baby as usual, over the collection bag.

Note: It may take a few tries to get the bag correctly into place. Check the infant frequently and change the bag after the infant has urinated. Drain the urine into the special container provided by your health care provider. Take the container to the laboratory or your health care provider as soon as possible.

Extra collection bags may be necessary if the urine sample is being taken from an infant.

Your doctor may tell you to stop taking any medicines that may affect the test results. NEVER stop taking any medicine without first talking to your doctor.

Drugs that can affect test measurements include:

• Aminosalicylic acid
• Birth control pills
• Barbiturates
• Chloral hydrate
• Chlorpropamide
• Ethyl alcohol
• Griseofulvin
• Morphine
• Phenazopyridine
• Procaine
• Sulfonamides

The test will feel the same as normal urination.

Your doctor will order this test if you have signs of porphyria or other disorders that can cause abnormal urine porphyrins.

Normal results vary. In general, for a 24-hour urine test, the range is about 50 - 300 mg (milligrams).

Abnormal results may be due to:

• Liver cancer
• Hepatitis
• Lead poisoning
• Porphyria (several types)

Anderson K. The porphyrias. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 229.

McPherson R, Ben-Ezra J, Zhao S. Basic examination of urine. In: McPherson RA, Pincus MR. Henry's Clinical Diagnosis and Management by Laboratory Methods. 21st ed. Philadelphia, Pa: Saunders; 2006:chap 27.