Porphyria Educational Services

PORPHYRIA EDUCATIONAL SERVICES BULLETIN
Vol. 1 No. 52                           December 19, 1999
FOCUS:  Porphyrin Red Blood Cell Testing


Another of the tests for porphyria is that of the porphyrin red blood cell.

Often it is given in conjunction with a 24 porphyrin urine test.
Other names for this tests are: uroporphyrin levels; protoporphyrin
levels; porphyrins, total; coproporphyrin levels


Porphyrins are pigments found in both animal and plant life.
The Nobel Laureate, Hans Fischer in 1930,  called porphyrins that which
"make the grass green, and the blood red".

Coproporphyrin, protoporphyrin, and uroporphyrin are three porphyrins
related to red blood cells that can normally be measured in small
amount in the human blood stream.

Protoporphyrin is normally found in highest quantity and thus this
test is occasionally known as the PROTO test.

Additional tests must be performed to know the levels of specific
porphyrins.


The test is undertaken by drawing blood  from a vein (venipuncture).

The point of entry is often  from the inside of the elbow or the back of the
hand.
The puncture site is cleaned with antiseptic, and a tourniquet (an elastic
band)
or blood pressure cuff is placed around the upper arm to apply pressure
and restrict blood flow through the vein.

When this is done it  causes veins below the tourniquet to distend
(fill with blood).

A needle is inserted into the vein, and the blood is collected in
an air-tight vial or a syringe.

Midway through the  procedure, the tourniquet is removed so that
the blood  circulation cam resume.  Once the blood has been collected,
 the needle is removed, and the puncture site is covered to stop any
bleeding.

The sample is then placed in ice and taken immediately to the laboratory.

Sometimes when the needle is inserted to draw blood, it is found that
some people will feel moderate pain. The majority of people will  feel only
a
prick or stinging sensation. Afterward, there may be some throbbing.

The test is performed do that physicians can diagnose porphyrin
disorders involving red blood cells.


This test specifically measures total porphyrin levels, but reference
 values for the individual components are also included:

The normal value ranges are as follows:

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

It must be remembered that when you undertake this test you must follow
the dietary restrictions. If you do not follow the dietary restrictions it
will often  alter and invalidate test results.


\When you test results show abnormal increased levels of
coproporphyrins, it may indicate  congenital erythropoietic porphyria,
as well as sideroblastic anemia.


Increased protoporphyrin levels may indicate that the patients has
infection, thalassemia, sideroblastic anemia, lead poisoning,
iron deficiency anemia, erythropoietic anemia , or increased erythropoiesis.


Increased uroporphyrin levels iften indicate erythropoietic protoporphyria.


This test must be ordered by your physician.
In order to prepare for the test you must be fasting for 12 to 14 hours
prior
to the time of  this test. i You may drink water right before the test.

If you have any carbohydrate intake, you will compromise your test.