Rates of neutropenia vary based on a child’s ethnicity and where they live in the world. Globally, the condition is most prevalent among children in North Africa and Arab nations, affecting about 15.4% of children under 6 years of age. Neutropenia is more common in infancy and early childhood with rates dropping as a child grows.
In the United States, the rate of neutropenia is 7.2% for children under 2 years old and 3.7% for those aged 3 to 5. It drops to 2.3% for 6 to 8-year-olds.
This article discusses the types of pediatric neutropenia, their causes, and their diagnosis. It also looks at treatment options and prognosis for children with this condition.
Types of Pediatric Neutropenia
Several types of neutropenia can affect children. These include:
Acute neutropenia: The most common reason for pediatric neutropenia is a viral infection. Neutropenia happens as the body uses up white blood cells to fight the infection. This is a temporary condition. White blood cell levels should return to normal once the child recovers. This usually takes six weeks or less. Cyclic neutropenia: This type can affect children and adults. More than one person in a family is likely to have it. It is called cyclic because low neutrophil counts are only present for 3 to 6 days every three weeks (on average). In children, the condition usually begins to improve after puberty. Congenital neutropenia: This type of neutropenia is inherited and tends to be the most severe. It is the most common type in children younger than 4 and is usually diagnosed when the child develops a life-threatening bacterial or fungal infection. Chronic idiopathic neutropenia: This type is referred to as idiopathic because there is no known cause. Autoimmune neutropenia: This type may also be called chronic benign neutropenia of childhood. The bone marrow makes the neutrophils normally, but the body incorrectly makes antibodies to neutrophils that mark them for destruction. Autoimmune neutropenia is often diagnosed around 7 to 9 months of age. Fortunately, it disappears in most children on its own within two years.
Causes
In children, neutropenia can have a number of causes. These can include:
A bacterial infection such as tuberculosis A viral infection such as mononucleosis An autoimmune condition that causes the immune system to mistakenly destroy neutrophils Inadequate bone marrow production, which can happen as a result of a condition such as aplastic anemia Radiation therapy or chemotherapy as a part of cancer treatment can destroy neutrophils
Sometimes, the cause of neutropenia isn’t known. When this happens the condition is referred to as idiopathic neutropenia.
Symptoms
Most children with neutropenia have no symptoms. That is because, despite the exceptionally low neutrophil count, serious infections are rare.
However, you may notice that your child gets sick more often. They may have more colds, ear infections, or sinus infections. Some children with neutropenia also get urinary tract infections or infections of the reproductive tract. Some also experience mouth sores or skin infections.
In these cases, neutropenia may be discovered on a complete blood count (CBC) done to diagnose an infection.
Diagnosis
As with other forms of neutropenia, the first diagnostic test is a complete blood count (CBC). The absolute neutrophil count (ANC) is typically below 1000 cells per microliter and can be below 500. Usually, the hemoglobin and platelet count are normal.
A peripheral blood smear may also be done. This is an examination of the blood cells under a microscope. Although the number of neutrophils is low, they usually look normal.
Next, your physician will likely obtain CBCs twice weekly for at least 6 weeks. This is done to ensure that your child does not have cyclic neutropenia.
In some patients, anti-neutrophil antibodies are never identified. In these cases, if the age and presentation fit the picture of autoimmune neutropenia, the diagnosis is presumed.
In rare cases, a bone marrow examination might be necessary to rule out other causes of neutropenia. This typically occurs in children whose presentation and infections don’t fit the typical picture of pediatric autoimmune neutropenia.
Treatment
There is no specific treatment for neutropenia. If a child with the condition develops a fever, they will typically undergo a CBC. Your child’s healthcare provider may also order a blood culture. During this test, a blood sample is placed in a glass bottle and checked for bacteria. Your child will also receive at least one dose of antibiotics.
If the ANC is less than 500 cells/mL, your child will likely be admitted to the hospital on IV antibiotics for observation. Sometimes filgrastim (G-CSF) can be used during active infections to stimulate the release of neutrophils from bone marrow to blood circulation.
If your child looks well and the ANC is greater than 1,000 cell/mL, they will likely be sent home for outpatient follow-up.
If your child has autoimmune neutropenia, the anti-neutrophil antibodies will spontaneously disappear and the neutrophil count will return to normal. Spontaneous recovery occurs by 5 years of age, with neutropenia lasting on average 20 months.
Medications used for other immune blood disorders (ITP, AIHA) like steroids and intravenous immune globulin (IVIG) are not as successful in autoimmune neutropenia.
Prognosis
Most children with neutropenia do not become seriously ill. Still, your healthcare provider will want to be cautious. Any fever or infection in a child with neutropenia should be evaluated by a healthcare provider.
Most of the time, neutropenia is a disease of childhood that resolves on its own before adulthood.
Summary
Pediatric neutropenia is a potentially serious condition that affects bacteria-fighting white blood cells called neutrophils. Children with this condition have low levels of neutrophils in their blood. This can lead to more frequent infections, which can sometimes be life-threatening.
Children with neutropenia may be diagnosed after a complete blood count is done as part of a diagnosis for a condition such as an ear or skin infection. Children with neutropenia are carefully monitored and may receive antibiotics or be admitted to the hospital for observation when they develop a fever.
Fortunately, neutropenia often resolves as a child gets older.
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