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 for many types of infection.

有四种类型的嗜中性白血球减少症:

Congenital

Congenital neutropenia is present at birth. Severe congenital neutropenia is also called Kostmann syndrome. It causes very low neutrophil levels and in some cases, complete lack of neutrophils. This puts infants and young children at risk for serious infections.

Cyclic

Cyclic neutropenia is present at birth and causes neutrophil counts to vary in a 21-day cycle. A period of neutropenia may last a few days, followed by normal levels for the rest of the cycle. The cycle then begins again.

Autoimmune

With autoimmune neutropenia, your body makes antibodies that fight your neutrophils. These antibodies kill the neutrophils, causing neutropenia.

Autoimmune neutropenia is most common in infants and young children, with the average age of diagnosis between 7 and 9 months .

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:

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 3-week cycles. Infections can increase 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:

Other causes include:

  • Shwachman-Diamond syndrome, which is an inherited condition affecting many organs and often characterized by bone marrow and pancreatic failure
  • 糖原累积病1型b,这是罕见的inherited disorder that affects blood sugar levels
  • leukemia
  • viral illnesses
  • severe aplastic anemia
  • Fanconi anemia
  • conditions that affect bone marrow
  • infections, both viral and bacterial, includingHIV,hepatitis,tuberculosis, andLyme disease
  • deficiencies in certain vitamins and minerals, includingB12,folic acid, andcopper

According to theU.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 certain conditions, such as:

  • cancer
  • leukemia
  • a weakened immune system

Chemotherapy and radiation therapy also raise the risk.

Idiopathic neutropenia affects people of all ages, but people over 70 are at higher risk. Men and women are at equal risk.

Your doctor can use these tests to diagnose neutropenia:

  • Complete blood count (CBC).This testmeasures neutrophil counts. Intermittent CBC tests can help your doctor check for changes in neutrophil count three times per week for 6 weeks.
  • Antibody blood test.This test checks for autoimmune neutropenia.
  • Bone marrow aspirate.This proceduretests bone marrow cells.
  • Bone marrow biopsy.This involves testing a piece of the bony part of bone marrow.
  • Cytogenetic and molecular testing.This testing helps your healthcare provider study the structures of the 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 (under the skin) injection. The treatment sometimes includes bone marrow transplants. This is usually when leukemia is present or G-CSF doesn’t work.

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

Neutropenia can last for months or years. It’s called acute when it lasts for fewer than 3 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, about40 percentof people with congenital neutropenia have decreased bone density. This puts them at a higher risk forosteoporosis.

About 20 percent have leukemia or blood and bone marrow disease in adolescence.

Treatment of neutropenia emphasizes helping you live a normal life. Managing it involves:

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

No specific prevention for neutropenia is known. However, theNational Neutropenia Networkadvises 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 with your doctor before traveling out of the country.

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