Aplastic anemia

What is aplastic anemia?

Aplastic anemia occurs when the bone marrow produces too few of all types of blood cells: red cells, white cells and platelets. A reduced number of red blood cells causes the hemoglobin (a type of protein in the red blood cells that carries oxygen to the tissues of the body) to drop. A reduced number of white blood cells causes the patient to be susceptible to infection. A reduced number of platelets can cause the blood not to clot as easily.

What causes aplastic anemia?

Aplastic anemia in children has multiple causes. Some of these causes are idiopathic, meaning they occur sporadically for no known reason. Other causes are secondary, resulting from a previous illness or disorder.

Nearly 50 to 75 percent of childhood cases of aplastic anemia occur sporadically for no known reason. Acquired causes, however, may include:

• History of specific infectious diseases such as hepatitis, Epstein-Barr virus (EBV), cytomegalovirus (CMV), parvovirus B19, or human immunodeficiency virus (HIV).
• History of taking certain medications.
• Exposure to certain toxins such as heavy metals.
• Exposure to radiation.
• History of an autoimmune disease such as lupus.

Children may also inherit a disorder that predisposes them to developing aplastic anemia. Some disorders that are known to predispose a child to aplastic anemia include the following:

• Fanconi anemia.
• Dyskeratosis congenita.
• Shwachman-Diamond syndrome.
• Reticular dysgenesis.
• Amegakaryocytic thrombocytopenia.
• Familial aplastic anemias.

What are the symptoms of severe aplastic anemia?

The following are the most common symptoms of severe aplastic anemia. However, each child may experience symptoms differently. Symptoms may include:

• Headache.
• Dizziness.
• Nausea.
• Shortness of breath.
• Bruising.
• Lack of energy or tiring easily (fatigue).
• Abnormal paleness or lack of color of the skin.
• Blood in stool.
• Nosebleeds.
• Bleeding gums.
• Fevers.
• Sinus tenderness.
• Enlarged liver or spleen.
• Oral thrush (white patches on a red, moist, swollen surface, occurring anywhere in the mouth).

The symptoms of severe aplastic anemia may resemble other blood disorders or medical problems. Always consult your child's physician for a diagnosis.

How is aplastic anemia diagnosed?

In addition to a complete medical history and physical examination of your child, diagnostic procedures for aplastic anemia may include:

• Blood tests.
• Bone marrow aspiration and biopsy - marrow may be removed by aspiration or a needle biopsy under local anesthesia. In aspiration biopsy, a fluid specimen is removed from the bone marrow. In a needle biopsy, marrow cells (not fluid) are removed. These methods are often used together.

Treatment for aplastic anemia

Specific treatment for aplastic anemia will be determined by your child's physician based on:

• Your child's age, overall health and medical history.
• The extent of the anemia.
• Cause of the anemia.
• Your child's tolerance for specific medications, procedures or therapies.
• Expectations for the course of the anemia.
• Your opinion or preference.

Aplastic anemia is a serious illness and treatment usually depends on the underlying cause. For certain causes, recovery can be expected after treatment, however, relapses can occur. To treat the low blood counts, initially treatment is usually supportive, meaning that it is necessary to treat the symptoms but not possible to cure the disease. Supportive therapy may include:

• Blood transfusion (both red blood cells and platelets).
• Preventive antibiotic therapy.
• Meticulous handwashing.
• Special care to food preparation (such as only eating cooked foods).
• Avoiding construction sites which may be a source of certain fungi.
• Medications (to stimulate the bone marrow to produce cells).
• Immunosuppressive therapy.
• Hormones.

Preventing infection and/or bleeding

Activities which put children with aplastic anemia at risk for infection or bleeding should be avoided. These activities include the following:

• Dental work (until your child's white blood cell counts rise).
• Contact sports (i.e., football, hockey, skiing, or rollerblading).
• Traveling to high altitudes (children with a low red blood cell count will have increased fatigue and need for oxygen in high altitudes).

If a child has a relapse of aplastic anemia, additional treatment (including a possible bone marrow transplant) may be necessary.