Neutropenia: Definition and Patient Education

Neutropenia is a blood condition characterized by low levels of neutrophils, which are white blood cells that protect your body from infections. Without enough neutrophils, your body can't fight off bacteria. Having neutropenia increases your risk of many types of infection.

Four types of neutropenia exist:

Congenital

Congenital neutropenia is present at birth. 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

Cyclic neutropenia is present at birth. 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.

Autoimmune

In autoimmune neutropenia, your body makes antibodies that fight your neutrophils. These antibodies kill the neutrophils, and this causes neutropenia. Autoimmune neutropenia develops later in life.

Idiopathic

Idiopathic neutropenia develops any time in life and can affect anyone. The cause is unknown.

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

Typical symptoms include:

• a fever
• pneumonia
• sinus infections
• otitis media, or an ear infection
• gingivitis, or gum inflammation
• omphalitis, or navel infection
• skin abscesses

Severe congenital neutropenia can have serious symptoms. The symptoms often include bacterial infections. These infections can grow on the skin, and in the digestive and respiratory systems.

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

The symptoms of autoimmune and idiopathic neutropenia include infections. They're usually not as severe as those in congenital forms.

Neutropenia can be triggered by:

• chemotherapy
• radiation therapy
• the use of certain drugs

Other causes include:

• Shwachman-Diamond syndrome, which is an inherited condition affecting many organs of the body and often characterized by bone marrow and pancreatic failure
• glycogen-storage disease type 1b, which is a rare inherited disorder that affects blood sugar levels
• leukemia
• viral illnesses
• severe aplastic anemia
• Fanconi anemia
• conditions that affect bone marrow

According to the U.S. National Library of Medicine, most people with severe congenital neutropenia have no family history of the condition.

The risk of neutropenia is increased by conditions, such as:

• cancer
• leukemia
• 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.

Your doctor can use these tests to diagnose neutropenia:

• A complete blood count (CBC) measures neutrophil counts.
• Intermittent CBC tests can help your doctor check for changes in neutrophil count three times per week for six weeks.
• An antibody blood test checks for autoimmune neutropenia.
• Your doctor can use bone marrow aspirate to test bone marrow cells.
• A bone marrow trephine biopsy tests a piece of the bony part of bone marrow.
• Cytogenetic and molecular testing study the structures of cells.

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 as a daily subcutaneous injection. The treatment sometimes includes bone marrow transplants. This is usually when leukemia is present or G-CSF fails.

The following therapies can also treat infections that occur due to the disorder:

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

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

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

Having severe congenital neutropenia increases your risk for other conditions. According to the U.S. National Library of Medicine, about 40 percent of people with congenital neutropenia have decreased bone density. This puts them at a higher risk for osteoporosis. About 20 percent have leukemia or blood and bone marrow disease in adolescence.

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

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

No specific prevention for neutropenia is known. However, the National Neutropenia Network 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°F (38.5°C).
• Wash your 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.

These preventive lifestyle measures can help you to reduce potential complications of neutropenia. Talk to your doctor about any symptoms that arise, and always know how to reach your doctor and hospital.