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Aplastic anemia

Aplastic anemia is a condition where the bone marrow does not produce enough, or any, new cells to replenish the blood cells. more...

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The term 'aplastic' refers to the inability of the marrow to function properly. Anemia is the condition of having fewer blood cells than normal, or fewer than needed to function properly. Typically, anemia refers to low red blood cell counts, but aplastic anemia patients have lower counts on all three blood cell types: red blood cells, white blood cells, and platelets.

Causes

One known cause is an autoimmune disorder, where the white blood cells attack the bone marrow.

In many cases, the etiology is impossible to determine, but aplastic anemia is sometimes associated with exposure to substances such as benzene or to the use of certain drugs, including chloramphenicol and phenylbutazone.

Signs and symptoms

  • Anemia with malaise, pallor and associated symptoms
  • Thrombocytopenia (low platelet counts), leading to increased risk of hemorrhage and bruising
  • Leukopenia (low white blood cell count), leading to increased risk of infection

Diagnosis

The diagnosis can only be made on bone marrow biopsy. Before this procedure is undertaken, a patient will generally have had other blood tests to find diagnostic clues, including a full blood count, renal function and electrolytes, liver enzymes, thyroid function tests, vitamin B12 and folic acid levels.

Treatment

Treating aplastic anemia involves suppression of the immune system, an effect achieved by daily medicine intake, or, in more severe cases, a bone marrow transplant, a curing but risky procedure. Bone marrow transplant replaces the old bone marrow cells with new ones from a donor, giving the patient a new immune system. There is a risk that the newly created white blood cells may attack the rest of the body ("graft-versus-host disease").

Steroids are generally ineffective, and many patients eventually receive ciclosporin or mild chemotherapy to silence the immune system. This usually happens with the agents cyclophosphamide and vincristine. Antibodies (anti-thymocyte globulin and anti-lymphocyte globulin) may be used in combination with them.

Follow-up

Regular full blood counts are required to determine whether the patient is still in a state of remission.

10-33% of all patients develop the rare disease paroxysmal nocturnal hemoglobinuria (PNH, anemia with thrombopenia and/or thrombosis), which has been explained as an escape mechanism by the bone marrow against destruction by the immune system. Flow cytometry testing is probably warranted in all PNH patients with recurrent aplasia.

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Anemia
From Gale Encyclopedia of Alternative Medicine, 4/6/01 by Paula Ford-Martin

Definition

Anemia is a condition characterized by abnormally low levels of healthy red blood cells or hemoglobin.

Description

The tissues of the human body need a regular supply of oxygen to stay healthy. Red blood cells, which contain hemoglobin that allows them to deliver oxygen throughout the body, live for only about 120 days. When they die, the iron they contain is returned to the bone marrow and used to create new red blood cells. Anemia can develop when heavy bleeding causes significant iron loss. It also occurs when something happens to slow down the production of red blood cells or to increase the rate at which they are destroyed.

Anemia can be mild, moderate, or severe enough to lead to life-threatening complications. Over 400 different types of anemia have been identified. Many of them are rare. More common anemia types include:

  • iron deficiency anemia
  • folic acid deficiency anemia
  • vitamin B12 deficiency anemia
  • vitamin C deficiency anemia
  • autoimmune hemolytic anemia
  • hemolytic anemia
  • sickle cell anemia
  • aplastic anemia
  • anemia of chronic disease

Causes & symptoms

Anemia is caused by bleeding, decreased red blood cell production, or increased red blood cell destruction. Poor diet can contribute to vitamin deficiency and iron deficiency anemia, in which fewer red blood cells are produced. Hereditary disorders and certain diseases can cause increased blood cell destruction. However, excessive bleeding is the most common cause of anemia, and the speed with which blood loss occurs has a significant effect on the severity of symptoms. Chronic blood loss may be caused by:

  • heavy menstrual flow
  • hemorrhoids
  • nosebleeds
  • cancer
  • gastrointestinal tumors
  • diverticulosis
  • polyposis
  • stomach ulcers
  • long-term alcohol abuse

Acute blood loss is usually the result of:

  • childbirth
  • injury
  • ruptured blood vessel
  • surgery

Iron deficiency anemia

Iron deficiency anemia is the most common form of anemia in the world. In the United States, iron deficiency anemia affects about 240,000 toddlers between one and two years of age and 3.3 million women of childbearing age. This condition is less common in older children and in adults over 50, and it rarely occurs in teenage boys and young men.

The onset of iron deficiency anemia is gradual. The deficiency begins when the body loses more iron than it gains from food and other sources. Because depleted iron stores cannot meet the red blood cells' needs, fewer red blood cells develop. In this early stage of anemia, the red blood cells look normal, but they are reduced in number. Then the body tries to compensate for the iron deficiency by producing more red blood cells, which are characteristically small in size.

Weakness, fatigue, and a run-down feeling may be signs of mild anemia. Other signs include skin that is pasty or sallow, or lack of color in the creases of the palm, gums, nail beds, or lining of the eyelids. Someone who is weak, tires easily, is often out of breath, and feels faint or dizzy may be severely anemic. Other symptoms of anemia are:

  • angina pectoris (chest pain)
  • headache
  • inability to concentrate and/or memory loss
  • inflammation of the mouth (stomatitis) or tongue (glossitis)
  • insomnia
  • irregular heartbeat
  • loss of appetite
  • nails that are dry, brittle, or ridged
  • rapid breathing
  • sores in the mouth, throat, or rectum
  • sweating
  • swelling of the hands and feet
  • thirst
  • tinnitus (ringing in the ears)
  • unexplained bleeding or bruising
  • pica (a craving to chew ice, paint, or dirt)

Folic acid deficiency anemia

Folic acid deficiency anemia is the most common type of megaloblastic anemia, in which red blood cells are bigger than normal. It is caused by a deficiency of folic acid, a vitamin that the body needs to produce normal cells.

Folic acid anemia is especially common in infants and teenagers. Although this condition usually results from a dietary deficiency, it is sometimes due to an inability to absorb enough folic acid from foods such as:

  • eggs
  • fish
  • green vegetables
  • meat
  • milk and cheese
  • mushrooms
  • yeast

Smoking raises the risk of developing this condition by interfering with the absorption of vitamin C, which the body needs to absorb folic acid. Folic acid anemia can be a complication of pregnancy, when a woman's body needs eight times more folic acid than it does otherwise.

Vitamin B12 deficiency anemia

Less common in this country than folic acid anemia, vitamin B12 deficiency anemia is another type of megaloblastic anemia that develops when the body does not absorb enough of this nutrient. Necessary for the creation of red blood cells, B12 is found in meat and vegetables.

Large amounts of B12 are stored in the body, so this condition may not become apparent until as long as four years after B12 absorption slows down or stops. The resulting drop in red blood cell production can cause:

  • loss of muscle control
  • loss of sensation in the legs, hands, and feet
  • soreness or burning of the tongue
  • weight loss
  • yellow-blue color blindness

The most common form of B12 deficiency is pernicious anemia. Since most people who eat meat or eggs get enough B12 in their diets, a deficiency of this vitamin usually means that the body is not absorbing it properly. This can occur among people who have had intestinal surgery or those who do not produce adequate amounts of intrinsic factor, a chemical secreted by the stomach lining that combines with B12 to help its absorption in the small intestine. Symptoms of pernicious anemia include problems with movement or balance, a slick tongue, tingling in the hands and feet, confusion, depression, and memory loss. Pernicious anemia can also damage the spinal cord. A doctor should be notified whenever symptoms of this condition occur.

Pernicious anemia usually strikes people 50-60 years of age. Eating disorders or an unbalanced diet increases the risk of developing pernicious anemia. So do diabetes mellitus , gastritis, stomach cancer, stomach surgery, thyroid disease, and family history of pernicious anemia.

Vitamin C deficiency anemia

A rare disorder that causes the bone marrow to manufacture abnormally small red blood cells, vitamin C deficiency anemia results from a severe, long-standing dietary deficiency.

Hemolytic anemia

Some people are born with hemolytic anemia. Some acquire this condition, in which infection or antibodies destroy red blood cells more rapidly than bone marrow can replace them.

Hemolytic anemia can enlarge the spleen and accelerate the destruction of red blood cells (hemolysis). Other complications of hemolytic anemia may include pain, shock, gallstones, and other serious health problems.

Thalassemias

An inherited form of hemolytic anemia, thalassemia stems from the body's inability to manufacture as much normal hemoglobin as it needs. There are two categories of thalassemia, depending on which of the amino acid chains is affected. (Hemoglobin is composed of four chains of amino acids.) In alpha-thalassemia, there is an imbalance in the production of the alpha chain of amino acids ; in beta-thalassemia, there is an imbalance in the beta chain. Alpha-thalassemias most commonly affect blacks (25% have at least one gene); beta-thalassemias most commonly affect people of Mediterranean and Southeast Asian ancestry.

Characterized by production of red blood cells that are unusually small and fragile, thalassemia only affects people who inherit the gene for it from each parent (autosomal recessive inheritance).

Autoimmune hemolytic anemia

Warm antibody hemolytic anemia is the most common type of this disorder. This condition occurs when the body produces autoantibodies that coat red blood cells. The coated cells are destroyed by the spleen, liver, or bone marrow.

Warm antibody hemolytic anemia is more common in women than in men. About one-third of patients who have warm antibody hemolytic anemia also have lymphoma, leukemia, lupus, or connective tissue disease.

In cold antibody hemolytic anemia, the body attacks red blood cells at or below normal body temperature. The acute form of this condition frequently develops in people who have had pneumonia, mononucleosis, or other acute infections. It tends to be mild and short-lived, and disappears without treatment.

Chronic cold antibody hemolytic anemia is most common in women and most often affects those who are over 40 and have arthritis. This condition usually lasts for a lifetime, generally causing few symptoms. However, exposure to cold temperatures can accelerate red blood cell destruction, causing fatigue, joint aches, and discoloration of the arms and hands.

Sickle cell anemia

Sickle cell anemia is a chronic, incurable condition that causes the body to produce defective hemoglobin, which forces red blood cells to assume an abnormal crescent shape. Unlike normal oval cells, fragile sickle cells cannot hold enough hemoglobin to nourish body tissues. The deformed shape makes it hard for sickle cells to pass through narrow blood vessels. When capillaries become obstructed, a life-threatening condition called sickle cell crisis is likely to occur.

Sickle cell anemia is hereditary. It almost always affects people of African or Mediterranean descent. A child who inherits the sickle cell gene from each parent will have the disease, but a child who inherits the gene from only one parent will carry the sickle cell trait, but will not have the disease.

Aplastic anemia

Sometimes curable by bone marrow transplant, but potentially fatal, aplastic anemia is characterized by decreased production of red and white blood cells and platelets (disc-shaped cells that allow the blood to clot). This disorder may be inherited or acquired as a result of recent severe illness, long-term exposure to industrial chemicals, or use of anticancer drugs and certain other medications.

Anemia of chronic disease

Cancer, chronic infection or inflammation, and kidney and liver disease often cause mild or moderate anemia. Chronic liver failure generally produces the most severe symptoms.

Diagnosis

Personal and family health history may suggest the presence of certain types of anemia. Laboratory tests that measure the percentage of red blood cells or the amount of hemoglobin in the blood are used to confirm diagnosis and determine which type of anemia is responsible for a patient's symptoms. X rays and examinations of bone marrow may be used to identify the source of bleeding.

Treatment

Anyone who has anemia caused by poor nutrition should modify his or her diet to include more vitamins, minerals, and iron. Foods such as lean red meats, dried beans and fruits, liver, poultry, and enriched breads and cereals are all good sources of iron. In addition, eating foods rich in vitamin C such as citrus fruits and juices can promote the absorption of iron.

Patients diagnosed with iron-deficiency anemia should undergo a thorough physical examination and medical history to determine the cause of the anemia, particularly if chronic or acute blood loss is suspected. The cause of a specific anemia will determine the type of treatment recommended.

Anemia due to nutritional deficiencies can usually be treated at home with iron supplements or self administered injections of vitamin B12. People with folic acid anemia should take oral folic acid replacements. Vitamin C deficiency anemia can be cured by taking daily supplements of vitamin C.

Many therapies for iron-deficiency anemia focus on adding iron-rich foods to the diet or on techniques to improve circulation and digestion. Iron supplementation, especially with iron citrate (less likely to cause constipation), can be given in combination with herbs that are rich in iron. Some examples of iron-rich herbs are dandelion (Taraxacum officinale), parsley (Petroselinum crispum), and nettle (Urtica dioica). The homeopathic remedy ferrum phosphoricum (iron sulfate) can also be helpful.

An iron-rich herbal tonic can also be made using the following recipe:

  • Soak one-half ounce of yellow dock root and one-half ounce dandelion root in 1 qt of boiled water for 4-8 hours.
  • Simmer until the amount of liquid is reduced to 1 cup.
  • Remove from heat and add one-half cup black strap molasses, mixing well.
  • Store in refrigerator; take one-quarter cup daily.

Other herbal remedies known to promote digestion are prescribed to treat iron-deficiency anemia. Gentian (Gentiana lutea) is widely used in Europe to treat anemia and other nutritionally-based disorders. The bitter qualities of gentian help stimulate the digestive system, making iron and other nutrients more available for absorption. This bitter herb can be brewed into tea or purchased as an alcoholic extract (tincture).

Other herbs recommended to promote digestion include:

  • anise (Pimpinella anisum)
  • caraway (Carum carvi)
  • cumin (Cuminum cyminum)
  • linden (Tilia spp.)
  • licorice (Glycyrrhiza glabra)

Traditional Chinese treatments for anemia include:

  • Acupuncture to stimulate a weakened spleen.
  • Asian ginseng (Panax ginseng) to restore energy.
  • Dong quai (Angelica sinensis) to control heavy menstrual bleeding.
  • A mixture of dong quai and Chinese foxglove (Rehmannia glutinosa) to clear a sallow complexion.
  • Astragalus (Astragalus membranaceus) to treat pallor and dizziness.

Allopathic treatment

Surgery may be necessary to treat anemia caused by excessive loss of blood. Transfusions of red blood cells may be used to accelerate production of red blood cells.

Medication or surgery may also be necessary to control heavy menstrual flow, repair a bleeding ulcer, or remove polyps (growths or nodules) from the bowels.

Patients with thalassemia usually do not require treatment. However people with a severe form may require periodic hospitalization for blood transfusions and/or bone marrow transplantation.

Sickle cell anemia

Treatment for sickle cell anemia involves regular eye examinations, immunizations for pneumonia and infectious diseases, and prompt treatment for sickle cell crises and infections of any kind. Psychotherapy or counseling may help patients deal with the emotional impact of this condition.

Vitamin B12 deficiency anemia

A life-long regimen of B12 shots is necessary to control symptoms of pernicious anemia. The patient may be advised to limit physical activity until treatment restores strength and balance.

Aplastic anemia

People who have aplastic anemia are especially susceptible to infection. Treatment for aplastic anemia may involve blood transfusions and bone marrow transplant to replace malfunctioning cells with healthy ones.

Anemia of chronic disease

There is no specific treatment for anemia associated with chronic disease, but treating the underlying illness may alleviate this condition. This type of anemia rarely becomes severe. If it does, transfusions or hormone treatments to stimulate red blood cell production may be prescribed.

Hemolytic anemia

There is no specific treatment for cold-antibody hemolytic anemia. About one-third of patients with warm-antibody hemolytic anemia respond well to large doses of intravenous and oral corticosteroids, which are gradually discontinued as the patient's condition improves. Patients with this condition who do not respond to medical therapy must have the spleen surgically removed. This operation controls anemia in about half of the patients on whom it is performed. Immune-system suppressants are prescribed for patients whose surgery is not successful.

Expected results

Folic acid and iron deficiency anemia

It usually takes three to six weeks to correct folic acid or iron deficiency anemia. Patients should continue taking supplements for another six months to replenish iron reserves and should have periodic blood tests to make sure the bleeding has stopped and the anemia has not recurred.

Pernicious anemia

Although pernicious anemia is considered incurable, regular B12 shots will alleviate symptoms and reverse complications. Some symptoms will disappear almost as soon as treatment begins.

Aplastic anemia

Aplastic anemia can sometimes be cured by a bone marrow transplant. If the condition is due to immunosuppressive drugs, symptoms may disappear after the drugs are discontinued.

Sickle cell anemia

Although sickle cell anemia cannot be cured, effective treatments enable patients with this disease to enjoy longer, more productive lives.

Thalassemia

People with mild thalassemia (alpha thalassemia trait or beta thalassemia minor) lead normal lives and do not require treatment. Those with severe thalassemia may require bone marrow transplantation. Genetic therapy is being investigated and may soon be available.

Hemolytic anemia

Acquired hemolytic anemia can generally be cured when the cause is removed.

Prevention

Inherited anemia cannot be prevented. Genetic counseling can help parents cope with questions and concerns about passing on disease-causing genes to their children.

Avoiding excessive use of alcohol, eating a balanced diet that contains plenty of iron-rich foods, and taking a daily multivitamin can help prevent anemia.

Methods of preventing specific types of anemia include:

  • Avoiding lengthy exposure to industrial chemicals and drugs known to cause aplastic anemia.
  • Not taking medication that has triggered hemolytic anemia and not eating foods that have caused hemolysis (breakdown of red blood cells).
  • Receiving regular B12 shots to prevent pernicious anemia resulting from gastritis or stomach surgery.

Key Terms

Aplastic
Exhibiting incomplete or faulty development.
Diabetes mellitus
A disorder of carbohydrate metabolism brought on by a combination of hereditary and environmental factors.
Hemoglobin
An iron-containing pigment of red blood cells composed of four amino acid chains (alpha, beta, gamma, delta) that delivers oxygen from the lungs to the tissues of the body.
Megaloblast
A large erythroblast (a red marrow cell that synthesizes hemoglobin).

Further Reading

For Your Information

Books

  • Fauci, Anthony S. et al., eds. Harrison's Principles of Internal Medicine. New York: McGraw-Hill, 1998.
  • Tierney, Lawrence M., Jr. Current Medical Diagnosis & Treatment, 1998. Stamford, Conn.: Appleton & Lange, 1998.

Gale Encyclopedia of Alternative Medicine. Gale Group, 2001.

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