What Is Neutropenia?

    Neutropenia is a blood condition. It occurs when neutrophil levels are low. Neutrophils are a type of white blood cell that protects your body from infections.

    Without enough neutrophils, your body can’t fight against bacteria. Having neutropenia increases your risk of many types of infection.

    Types of Neutropenia

    There are four types of neutropenia: congenital, cyclic, autoimmune, and idiopathic.

    Congenital neutropenia and cyclic neutropenia are present at birth. Autoimmune neutropenia and idiopathic neutropenia develop later in life.

    Severe congenital neutropenia is also called Kostmann syndrome. It causes very low neutrophil levels. In some cases, neutrophils are absent. This puts infants and young children at risk for serious infections.

    Cyclic neutropenia causes neutrophil counts to vary in a 21-day cycle. Neutrophil counts fall from normal to low. A period of neutropenia may last a few days. Normal levels follow for the rest of the cycle. The cycle then resets and begins again.

    In autoimmune neutropenia, your body makes antibodies that fight your own neutrophils. These antibodies kill the neutrophils, and this causes neutropenia.

    Idiopathic neutropenia can affect anyone. The cause is unknown.

    What Causes Neutropenia?

    Neutropenia can be triggered by chemotherapy, radiation therapy, and the use of certain drugs. Other causes are:

    • Shwachman-Diamond syndrome
    • glycogen-storage disease type 1b
    • leukemia
    • viral illnesses
    • severe aplastic anemia
    • Fanconi anemia
    • conditions that affect bone marrow

    Most people with severe congenital neutropenia have no family history of the condition (Genetics Home Reference).

    Who Is at Risk?

    The risk of neutropenia is increased by conditions such as cancer, leukemia, and a weakened immune system. Chemotherapy and radiation therapy also raise the risk.

    Idiopathic neutropenia affects patients of all ages, but people who are 70 years old or older are at higher risk. Men and women are at equal risk.

    What Are the Symptoms of Neutropenia?

    Neutropenia symptoms can range from mild to severe. The lower the level of neutrophils, the more intense the symptoms.

    Typical symptoms include:

    • fever
    • pneumonia
    • sinus infections
    • otitis media (ear infection)
    • gingivitis (gum inflammation)
    • omphalitis (navel infection)
    • skin abscesses

    Severe congenital neutropenia can have serious symptoms. Symptoms often include bacterial infections. These infections can grow on the skin. They also can be in the digestive and respiratory systems.

    Symptoms of cyclic neutropenia recur in three-week cycles. Infections can grow when neutrophil levels fall.

    Symptoms of autoimmune and idiopathic neutropenia include infections. They are usually not as severe as those in congenital forms.

    Diagnosing Neutropenia

    Your doctor can use these tests to diagnose neutropenia:

    • complete blood count/full blood count (CBC/FBC)­: measures neutrophil counts
    • intermittent CBC/FBC: studies changes in neutrophil count three times a week for six weeks
    • antibody blood test: tests for autoimmune neutropenia
    • bone marrow aspirate: tests bone marrow cells
    • bone marrow trephine biopsy: tests a piece of the bony part of bone marrow
    • cytogenetic and molecular testing: studies the structures of cells

    Treating Neutropenia

    Most cases of neutropenia can be treated with granulocyte-colony stimulating factors (G-CSF). This is a synthetic copy of the hormone that causes neutrophils to grow in the bone marrow. G-CSF can increase the number of neutrophils.

    G-CSF is usually given with a daily subcutaneous injection.

    Bone marrow transplants are sometimes used. This is usually when leukemia is present or if G-CSF fails.

    The following therapies can also treat infections caused by the disorder:

    • antibiotics
    • anti-inflammatory drugs
    • corticosteroids
    • cytokines
    • glucocorticoids
    • immunoglobulins
    • immunosuppressive drugs
    • white cell transfusions
    • vitamins

    Prognosis

    Neutropenia can last for months or years. It is called acute when it lasts less for than three months. When it lasts for a longer time, it is called chronic.

    Lower neutrophil levels can cause dangerous infections. These infections can be life-threatening when they are untreated.

    Severe congenital neutropenia creates a higher risk for other conditions. According to the U.S. National Library of Medicine, about 40 percent of patients with congenital neutropenia have decreased bone density. This puts them at a higher risk for osteoporosis.

    About 20 percent have leukemia or a blood and bone marrow disease in adolescence (Genetics Home Reference).

    Treatment of neutropenia emphasizes helping the patient live a normal life. Management requires:

    • annual bone marrow monitoring
    • monthly CBC/FBC tests
    • emotional support
    • psychological therapy

    Prevention

    There is no specific prevention for neutropenia. However, the National Neutropenia Network (NNN) advises the following to reduce complications:

    • Maintain good oral hygiene. Get regular dental exams, and use an antibacterial mouthwash.
    • Keep vaccinations current.
    • Get medical care for a fever above 101.3 degrees Fahrenheit (38.5 degrees Celsius).
    • Wash hands thoroughly.
    • Care for cuts and scrapes.
    • Use antibiotics and antifungals as directed.
    • Know how to reach your doctor and hospital.
    • Talk to your doctor before foreign travel.