Neutropenia

Overview

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 your child from infections. Neutropenia can be a very serious condition because without enough neutrophils, your child is susceptible to bacterial infections that can become life-threatening.

Among children, neutropenia has a number of causes, including:

• Inadequate bone marrow production due to other blood disorders such as aplastic anemia or cancer such as leukemia.
• Response to radiation therapy or chemotherapy, which destroys white cells. When this occurs, it could delay radiation or chemotherapy.
• Inadequate white cells because of an autoimmune disease.
• Bacterial infections, such as tuberculosis, or viral infections like mononucleosis.

Types of neutropenia

There are several forms of neutropenia:

• Congenital Neutropenia — This is a severe, inherited form of the disease and is most common among babies and very young children, rather than adults. Symptoms include frequent fevers, mouth sores, ear infections, pneumonia or rectal sores. If untreated, children may lose their teeth or develop severe gum infections. The most severe form of chronic congenital neutropenia is called Kostmann's syndrome.
• Cyclic Neutropenia — This condition 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. Symptoms include fever, illness and mouth ulcers. Children with cyclic neutropenia usually improve after puberty.
• Chronic Benign Neutropenia — This is a rare form of neutropenia that could result in life-threatening infections. This is the most common form in children under 4 years of age. The rate of infections decreases with age.

Signs & symptoms

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, sinuses and skin. Some patients experience painful mouth ulcers, gum infections, ear infections, periodontal disease or disease of the tissues surrounding the teeth and 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
• Unusual redness, swelling or warmth at the site of an injury

Diagnosis

If your child is experiencing symptoms of neutropenia, the doctor will request a complete blood count (CBC) to measure your child's neutrophil count. If the 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 also will undergo a blood test that looks for antibodies, to rule out the diagnosis of 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.

The bone marrow aspirate involves obtaining a small amount of bone marrow tissue and fluid. The sample is typically taken from the back of your child's pelvic bone. The bone is numbed and your child is given medication to reduce pain or possibly induce sleep. A needle is put through the outer bone into the marrow and a sample is removed. This will help determine what type of neutropenia your child has and to what extent it has developed.

Treatment

The two primary treatments for neutropenia are antibiotics to fight infection and drugs that help the bone marrow make neutrophils. In some cases, a bone marrow transplant may be an option.

Antibiotics

With severe neutropenia, children often develop fever. When they do, they are given antibiotics to fight infection and may need to be hospitalized. Repeated use of antibiotics, however, could have side effects such as the development of drug-resistant bacteria, diarrhea or enterocolitis, an inflammation of the intestines. Some antibiotics may have adverse effects on kidney and liver function.

Granulocyte Colony-Stimulating Factors

New drugs have been developed that help the bone marrow make neutrophils and restore the body's defense against infection. These drugs are known as granulocyte colony-stimulating factors. They help keep neutrophils in the blood above the danger level all or most of the time. The shorter the time the neutrophil count is low, the less chance there is of your child developing fever or infection. These drugs may decrease the number and severity of infections and reduce hospitalizations.

Bone Marrow Transplant

In some cases, a bone marrow transplant is an option. Bone marrow transplantation is a procedure in which healthy bone marrow is given to replace defective bone marrow. The transplant may be autologous, which means using the patient's own marrow that was removed and possibly treated to kill any defective cells; allogeneic, which means using marrow from a healthy "matched" donor, usually a brother or sister; or syngeneic, which means using marrow from an identical twin.