Neutropenia
Neutropenia is a condition where you have too few neutrophils in your blood, a type of white blood cell that helps your body fight infections. People with neutropenia are more vulnerable to infections because their body has fewer defenses against germs.
Table of contents
- What is neutropenia?
- Classification and severity levels
- Who is affected by neutropenia?
- What causes neutropenia?
- Symptoms and signs
- How is neutropenia diagnosed?
- Treatment approaches
- Preventing infections
- Living with neutropenia
What is neutropenia?
Neutropenia refers to having lower-than-normal levels of neutrophils in your blood[1]. Neutrophils are a type of white blood cell that your bone marrow primarily makes. These cells are crucial for your body’s immune system because they fight infections by destroying germs such as bacteria, viruses, and fungi[2].
When you don’t have enough neutrophils, your body finds it harder to fight germs and prevent infections. In severe cases, even bacteria that a healthy body normally tolerates, like those living in your mouth and intestines, can make you seriously ill[1].
Many people with neutropenia don’t experience any symptoms and may only discover they have the condition during a blood test done for another reason[3]. The main concern with neutropenia is the increased risk of infections, which can become life-threatening without proper treatment.
Classification and severity levels
Neutropenia is classified according to how many neutrophils are present in your blood. Healthcare providers measure this using the absolute neutrophil count (ANC), which is calculated based on the percentage of neutrophils in your white blood cell count[4].
A normal neutrophil count for adults is typically between 1,500 and 8,000 neutrophils per microliter of blood. When the count falls below 1,500, neutropenia is diagnosed. The severity levels are[1][2]:
- Mild neutropenia: 1,000 to 1,500 neutrophils per microliter
- Moderate neutropenia: 500 to 1,000 neutrophils per microliter
- Severe neutropenia: Less than 500 neutrophils per microliter
The risk of infection increases as the neutrophil count decreases. People with severe neutropenia face extremely high vulnerability to infections[4].
Neutropenia can also be classified by how long it lasts. Acute neutropenia is temporary or short-lasting, while chronic neutropenia persists for more than three months[1][17]. Additionally, congenital neutropenia is a condition you’re born with, while acquired neutropenia develops over time.
Who is affected by neutropenia?
Neutropenia can affect people of all ages, from babies to older adults. It’s especially common among people receiving cancer treatments. Researchers estimate that 50% of people receiving chemotherapy will develop neutropenia[1].
There is a specific form called benign ethnic neutropenia (BEN), which is most common in people of African, Middle Eastern, and West Indian descent. This is a chronic condition present from birth where neutrophil counts are below normal levels, but importantly, people with BEN are not at increased risk of infection[1].
Children can develop neutropenia, particularly congenital forms that appear in babies and very young children. The most severe form of chronic congenital neutropenia is called Kostmann’s syndrome[7].
What causes neutropenia?
Neutropenia occurs when your bone marrow doesn’t make enough neutrophils, when neutrophils break down too soon, or when they get destroyed[1]. There are many possible causes, which can be grouped into several categories.
Genetic and inherited conditions
Some forms of neutropenia are caused by genetic abnormalities that can be passed from parents to their biological children. Types of inherited neutropenia include benign ethnic neutropenia (BEN), cyclic neutropenia (where neutrophil levels drop periodically, often every three weeks), and severe congenital neutropenia[1][10].
Infections
Viral, bacterial, and parasitic infections can cause neutropenia. Common causes include HIV, hepatitis, tuberculosis, sepsis, Lyme disease, and mononucleosis[1][10].
Cancer and blood disorders
Cancer and other blood or bone marrow disorders, including leukemia, lymphoma, and myelodysplastic syndromes, can prevent your body from making enough healthy white blood cells[1].
Medications and treatments
Cancer treatments such as chemotherapy and radiation therapy can harm or destroy neutrophils and the bone marrow that makes them. This is a common side effect of these treatments. Medications for other conditions may also cause low neutrophil levels[1][6].
Nutritional deficiencies
Not having enough vitamins or minerals in your diet can lead to neutropenia. Important nutrients include vitamin B12, folate, and copper[1].
Autoimmune conditions
In autoimmune neutropenia, your immune system mistakenly targets and destroys your own neutrophils. This is the most common cause of neutropenia in infants and young children, though it can also occur in adults[10].
Symptoms and signs
Neutropenia itself often doesn’t cause any symptoms. Many people with mild neutropenia feel perfectly well and only learn they have the condition when blood tests are done for another reason[3][5].
The main problems come from infections, which people with neutropenia are more likely to develop. Common symptoms of infections in people with neutropenia include[4][5][10]:
- Fever (often the only symptom)
- Sore throat or painful swallowing
- Mouth sores or gum pain and swelling
- Skin abscesses or rashes
- Recurrent sinus infections or ear infections
- Cough or shortness of breath
- Pain or irritation in the rectal or genital areas
- Painful urination
- Unusual vaginal discharge
- Diarrhea
- Fatigue
In severe neutropenia or agranulocytosis (which means a virtual absence of neutrophils), symptoms can appear suddenly and may include high fever (often 40°C or higher), rapid pulse and breathing, low blood pressure, and signs of septic shock[4].
A particularly serious condition is febrile neutropenia, where someone with neutropenia develops a fever of 38°C or higher. This is a medical emergency that needs immediate attention because it can be life-threatening[5][8].
How is neutropenia diagnosed?
Neutropenia is diagnosed through blood tests. A complete blood count measures the number of different types of cells in your blood, including neutrophils. Because neutrophil levels can vary from day to day, if a blood test shows low levels, it usually needs to be repeated to confirm the diagnosis[3].
Once neutropenia is confirmed, your doctor will work to identify the cause. This may involve[4][12]:
- Taking a detailed medical history, including questions about medications, recent infections, and family history
- A thorough physical examination
- Additional blood tests to check for infections, autoimmune diseases, or vitamin deficiencies
- Bone marrow aspiration and biopsy to examine how neutrophils are being produced
- Genetic testing if a congenital form is suspected
The specific tests ordered will depend on your symptoms, medical history, and initial test results. Identifying the correct cause is vital for implementing appropriate treatment and monitoring[2].
Treatment approaches
Treatment for neutropenia depends on its cause, severity, and whether you have developed infections. In some cases, especially mild neutropenia without symptoms, careful monitoring may be all that’s needed[17].
Managing infections
If you develop an infection, prompt antibiotic treatment is essential. Fever in someone with neutropenia is often treated as an infection even before test results are available, because waiting could be dangerous[11]. Typical treatments include broad-spectrum antibiotics that work against many types of bacteria.
Growth factor therapy
Granulocyte colony-stimulating factor (G-CSF) is a medication that helps stimulate your bone marrow to produce more neutrophils. This treatment has been particularly effective for many forms of neutropenia, especially congenital types and chemotherapy-induced neutropenia[13][17].
Treating underlying causes
If neutropenia is caused by a medication, stopping that drug may be the most important step. For nutritional deficiencies, vitamin or mineral supplements may help. Autoimmune neutropenia might be treated with immunosuppressant medications[11].
Preventive measures
In some cases, especially when neutropenia is severe or prolonged, preventive antibiotics or antifungal medications may be prescribed to reduce the risk of infections[11][13].
Advanced treatments
For severe congenital forms that don’t respond to other treatments, hematopoietic stem cell transplantation may be considered[12].
Preventing infections
If you have neutropenia, taking steps to avoid infections is crucial. Good hygiene and careful daily habits can significantly reduce your risk[6][15].
Personal hygiene practices
- Wash your hands thoroughly and frequently with soap and water
- Use careful oral hygiene to prevent mouth infections, including gentle brushing and salt water rinses
- Take good care of your skin, treating any wounds or abrasions promptly
- Bathe or shower daily
Avoiding exposure to germs
- Stay away from people who are sick
- Avoid crowds during times when your neutrophil count is very low
- Do not allow fresh flowers or plants in your room if you’re hospitalized, as they can harbor bacteria and fungi[11]
- Be cautious around pets, particularly with animal waste
Food safety
When neutropenic, you may need to follow special food safety guidelines[21]:
- Prepare food with extra care
- Wash fruits and vegetables thoroughly
- Avoid raw meat, fish, or eggs
- Don’t eat foods that may carry harmful bacteria
- Practice good food hygiene to reduce your risk of food poisoning
Other precautions
- Avoid rectal temperature measurements and frequent rectal examinations[11]
- Use stool softeners if needed to prevent constipation
- Check with your healthcare team before having dental work or vaccinations
Living with neutropenia
Living with chronic neutropenia requires adjustments to daily routines and ongoing awareness of infection risks. However, many people with neutropenia, especially mild forms, can maintain a relatively normal lifestyle[17].
Monitoring and follow-up
Regular blood counts are often needed to track neutrophil levels over time. Your healthcare team will tell you how often you need testing. Keep track of your appointments and communicate any symptoms or concerns promptly[17].
School, work, and daily activities
Many children and adults with neutropenia attend school or work with few limitations. However, extra precautions may be needed during illness outbreaks. Open communication with teachers, school nurses, or employers can help create supportive environments[17].
When to seek medical help
Contact your doctor immediately if you develop[5]:
- Fever (38°C or higher)
- Signs of infection anywhere in your body
- Chills or sweating
- Mouth sores or sore throat
- Unusual pain or discomfort
If you have neutropenia and develop a fever, go to the nearest emergency department immediately. This is a medical emergency[5][8].
Emotional support
Living with a chronic condition can be challenging. Consider connecting with support groups, speaking with a counselor, or joining online communities for people with blood disorders[17]. Many hospitals and cancer centers offer resources for patients and families.
Looking ahead
The outlook for neutropenia varies greatly depending on its cause. Some types, like cyclic neutropenia in children, may improve after puberty. Others require long-term management. With proper treatment and precautions, many people with neutropenia live full, active lives[7][17].
Advances in treatment, particularly growth factor therapies, have significantly improved outcomes for people with neutropenia. Ongoing research continues to develop new approaches to diagnosis and treatment[13].
