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
- 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
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:
- 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
cyclic neutropenia recur in three-week cycles. Infections can grow when
neutrophil levels fall.
autoimmune and idiopathic neutropenia include infections. They are usually not as
severe as those in congenital forms.
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
- 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
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:
- anti-inflammatory drugs
- immunosuppressive drugs
- white cell transfusions
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
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.