Neutropenia

Neutropenia is a rare disorder that causes children to have lower than normal levels of neutrophils, a type of white blood cell that destroys bacteria in the blood and helps protect against infections. Neutropenia can be a very serious condition. Without enough neutrophils, the body is susceptible to bacterial infections that can become life-threatening.

There are several forms of neutropenia:

• Congenital neutropenia is a severe, inherited form of the disease and is most common among babies and very young children. The most severe form of chronic congenital neutropenia is called Kostmann's syndrome.
• Cyclic neutropenia occurs in both children and adults and is often present in several members of the same family. Cyclic neutropenia tends to occur every three weeks and last three to six days at a time. Children with cyclic neutropenia usually improve after puberty.
• Chronic benign neutropenia is the most common form of neutropenia in children younger than 4 years and can result in life-threatening infections. The rate of infections decreases with age.

Neutropenia has a number of causes among children, including:

• Inadequate bone marrow production due to another blood disorder (such as aplastic anemia) or cancer (such as leukemia).
• Radiation therapy or chemotherapy, which destroy white blood cells. When neutropenia occurs, radiation or chemotherapy may be delayed.
• An autoimmune disease causing inadequate white blood cells.
• Bacterial infections (such as tuberculosis) or viral infections (such as mononucleosis).

Children with neutropenia tend to develop infections easily because their white blood cell count is too low to ward off bacteria. Most infections occur in the lungs, mouth, throat, ears, sinuses and skin. Some patients experience painful mouth ulcers and disease of the tissue surrounding the teeth, as well as infections of the urinary tract, colon, rectum or reproductive tract.

Other symptoms include:

• Fever
• Shaking chills
• Sore throat
• Cough or shortness of breath
• Nasal congestion
• Diarrhea or loose bowels
• Burning during urination

If your child is experiencing symptoms of neutropenia, your doctor will request a complete blood count (CBC) to measure your child's neutrophil count. If your child’s neutrophil count is lower than normal, additional CBC tests will be performed to determine if there is a pattern of low neutrophil counts. Your child will also undergo a blood test that looks for antibodies in order to rule out other possible disorders.

If blood tests indicate that your child has neutropenia, your child's doctor will request a bone marrow examination, called a bone marrow aspirate, to confirm the diagnosis. In preparation for this procedure, your child will be given medicine to reduce pain and possibly induce sleep. A needle is then inserted through the back of the pelvic bone into the bone marrow. A small amount of bone marrow tissue and fluid is removed. This bone marrow sample will help determine what type of neutropenia your child has and to what extent it has developed.

Treatment for neutropenia includes:

• Antibiotics. Children with severe neutropenia often develop infections requiring antibiotics and possible hospitalization. Repeated use of antibiotics, however, can have side effects, such as the development of drug-resistant bacteria or diarrhea or enterocolitis, an inflammation of the intestines. Some antibiotics may also have adverse effects on kidney and liver function.
• Granulocyte colony-stimulating factors. These new medications help the bone marrow make neutrophils and restore the body's defense against infection. They help keep neutrophils above the danger level, reducing the chance of developing fever or infection. These drugs may also decrease the number and severity of infections and thereby reduce hospitalizations.
• Bone marrow transplant. In some cases, a bone marrow transplant is an option. Bone marrow transplantation is a procedure in which defective bone marrow is replaced with healthy bone marrow. The transplant may be autologous, meaning the patient's own marrow is removed and possibly treated to kill any defective cells; allogeneic, meaning healthy marrow from a matched donor, usually a sibling, is used; or syngeneic, meaning the healthy marrow from an identical twin is used.