Chronic benign neutropenia in adults

Neutropenia is a serious health disorder characterized by an abnormally low count of neutrophils. Neutrophils are the most abundant type of white blood cells in mammals, and the essential part of the human immune system. Normally, neutrophils make up anywhere from 50 to 70 percent of all blood mass in the body. They are the body's primary fighting mechanisms against infections, bacteria and foreign objects. Neutropenia can be an acute or chronic health problem.

Chronic neutropenia is diagnosed if the patient manifests the symptoms of the condition for more than 3 months. This condition is a subset of leukopenia as a whole, which is a condition described by a deficit in the number of white blood cells.

Normal levels of neutrophils are essential to prevent infections. Neutropenia is a relatively common disorder most often due to chemotherapy treatments, adverse drug reactions, or autoimmune disorders. The differential diagnosis of neutropenia is broad and encompasses congenital, infectious, rheumatologic, and iatrogenic causes.

Patients with a neutrophil count of less than 0.5 × 109/L (500 cells/?L) and signs or symptoms of infection require immediate hospitalization and prophylactic antibiotic therapy.
Severe neutropenia is most commonly due to cell destruction that is mediated by the immune system or hypoplasia, rather than sequestration.
Drugs are the most common cause of neutropenia, and medicines that can cause neutropenia should be discontinued whenever possible.
The appropriate use of cytokines is dependent on the underlying cause of neutropenia.

Epidemiological studies show a wide variation in the incidence of neutropenia among geographic regions, with an average annual incidence of 56.4 cases of neutropenia and 9.0 cases of agranulocytosis per million people in the United States.

Data from the International Agranulocytosis and Aplastic Anemia Study in Europe show an annual incidence of 6.2 cases of agranulocytosis per million people.

Both studies assessed only hospitalized patients with severe neutropenia due primarily to noncytotoxic drugs, infections, or immune disorders and excluded those patients who were treated with cytotoxic drugs. Male and nonwhite patients had an increased risk, and patients between 50 and 59 years of age had the highest risk.

✓ Fact confirmed: Severe neutropenia a diagnostic approach Hidayatullah G. Munshi and R. Bruce Montgomery; 2000 Apr

Causes of Neutropenia

Neutropenia usually results either from problems in the production of cells in the bone marrow or because these cells are destroyed somewhere in the body. Chronic benign neutropenia is the same condition as the autoimmune neutropenia that usually affects the children. Neutropenia in adults can be primary or secondary. The primary neutropenia, which rarely affects adults, is the milder condition that usually manifests in skin problems, stomatitis, gingivitis, upper respiratory tract infections, and otitis media. Primary neutropenia is a sole hematologic abnormality. Secondary neutropenia is a more severe condition, which is prevalent in adults. It results from autoimmune disease, infections, neurological diseases, drug exposure, or malignancy.

Signs and Symptoms of Neutropenia

In many cases, neutropenia goes undetected. Most of the patients never recognize their mild symptoms as a sign of a serious disease. The condition is usually diagnosed when the patient develops severe infection or sepsis. When the symptoms are present, they normally include fevers and frequent infections. Patients often suffer from mouth ulcers, diarrhea, burning sensation while urinating, strange and unexplained redness on the skin, pain and general body fatigue, swelling around the wounds and unusually sore throat. The condition can be diagnosed based on the full blood count, which can detect low neutrophils counts.

Treatment for Neutropenia

There is no cure for chronic neutropenia but many treatments are available and the choice of the treatment is based upon the cause of the disease. A condition called chronic benign neutropenia of childhood usually clears up by itself, but if it does not go away, patient may need certain drugs or other therapies. Growth factors like G-CSF, granulocyte-colony stimulating factor, or GM-CSF, granulocyte-macrophage colony-stimulating factor, are commonly used in therapy. If the disease results from an autoimmune reaction, the patient will probably have to use different corticosteroids or immunosuppressive therapies.