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Addison’s Anemia

Vitamin B12 Deficiency, Addison's Anemia, Congenital Pernicious Anemia, Combined Systems Disease

Facts

The blood that circulates throughout the body performs a number of critical functions. It delivers oxygen, removes carbon dioxide, and carries life-sustaining nutrients. By transporting long-distance messengers such as hormones, blood helps the various parts of the body communicate with each other. These important functions are performed by blood cells working in partnership with the liquid part of the blood (plasma). Most of the cells in it are red blood cells (erythrocytes). White blood cells (leukocytes) are also present in smaller numbers. Their role is to defend your body against foreign material, including infections, viruses, and fungi.

Anemia is a state that occurs when hemoglobin (an iron-protein compound in red blood cells that transports oxygen) is decreased and your body has too few red blood cells. When there are too few red blood cells due to a lack of vitamin B12, the condition is described as pernicious anemia. The term pernicious was adopted many years ago when there was no effective treatment and this condition was inevitably fatal. Today, excellent therapies are available and most people can lead a normal life with very little adverse effects.

Pernicious anemia can affect all racial groups, but the incidence is higher among fair-haired people, especially those whose ancestors came from Scandinavia or Northern Europe. It usually doesn’t appear before the age of 30, although a juvenile form of the disease can occur in children. About 4% of Canadians have insufficient blood levels of vitamin B12.

Alternative names for pernicious anemia are vitamin B12 deficiency (malabsorption), Addison’s anemia, and congenital pernicious anemia.

Causes

Pernicious anemia is caused by a deficiency of vitamin B12, which is needed for normal production of red blood cells. It is often hereditary. Risk factors include a history of autoimmune endocrine disorders, a family history of pernicious anemia, and Scandinavian or Northern European descent.

The meat and dairy products we eat are our primary sources of vitamin B12. However, except in strict vegetarians, pernicious anemia isn’t simply caused by not eating enough of these foods. Usually, it is because of a failure in the complex process the digestive tract must go through to absorb vitamin B12.

In order for vitamin B12 to be absorbed by the small intestine, the cells that line a part of the stomach must produce a substance called intrinsic factor (IF). This substance attaches itself to vitamin B12, and both are absorbed in combination into the lowest portion of the small bowel (ileum), just before the small bowel enters the colon. If the ileum is damaged or removed in the course of surgery, the intrinsic factor/vitamin B12 combination will not be absorbed. People with conditions like Crohn’s disease, who often have surgery to remove part of their ileum (the part of the small intestine where vitamin B12 is absorbed), should be screened for vitamin B12 deficiency and treated if needed.

Lack of intrinsic factor may also be congenital (present at birth). This form of pernicious anemia (called juvenile or congenital) is usually seen before a child is three years old. It is believed that only one parent needs to carry the gene for this disorder to pass it along to a child.

Less common causes of decreased B12 absorption include chronic pancreatitis, malabsorption syndromes, certain medications, and, very rarely, increased metabolism of B12 through longstanding hyperthyroidism. A very common cause of

B12 deficiency in the elderly is inadequate absorption of dietary B12.

Pernicious anemia is often also seen in combination with some autoimmune endocrine (gland) diseases such as type 1 diabetes, hypoparathyroidism, Addison’s disease, and testicular dysfunction.

Certain antacids, heart medication, or diabetes medication may contribute to B12 deficiency.

Treatment and Prevention

The amount of B12 stored in the body is directly related to the amount that is taken in. The main treatment for pernicious anemia is injections of vitamin B12. Calculating the required amount of vitamin B12 needed can be difficult, because it must also replace the B12 stored in the liver.

At first there may be 5 to 7 injections in a short span of time. This therapy usually makes a difference within 48 to 72 hours, so there shouldn’t be any need for a blood transfusion. Eventually, injections can be given once a month and will probably continue indefinitely. Vitamin B12 can also be given by mouth in very large doses (0.5 mg to 2 mg per day) for maintenance treatment, making injections unnecessary.

Pernicious anemia cannot be treated without the help of a doctor. However, a well-balanced diet is essential to provide other components necessary for healthy blood cell development, such as folic acid, iron, and vitamin C.

Vitamin B12 therapy must be maintained for life unless the underlying cause of the deficiency is corrected.

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