Autoimmune Neutropenia

Autoimmune Neutropenia

What is neutropenia? — Neutropenia is condition that happens when your blood does not have enough of the cells called "neutrophils." Neutrophils are a type of white blood cell. They help your body fight infections.
Blood is made up of different types of cells. These cells are made in the center of your bones, in a part called the bone marrow. Neutropenia can happen if:
Your bone marrow doesn't make enough neutrophils.
Something in your body (such a medicine you took, or your own immune system) destroys some of your neutrophils.
Some people with neutropenia have no symptoms. But people with severe neutropenia can get fevers and frequent infections.
What causes neutropenia? — Many different things can cause neutropenia, including:
Infections – Many different infections can cause neutropenia, including:
•Typhoid fever
•Tuberculosis
•Hepatitis B
•HIV, the virus that causes AIDS
Medicines – Many medicines can cause neutropenia. Some examples include:
•Medicines used to treat cancer, such as cyclophosphamide (brand name: Cytoxan) and doxorubicin (brand name: Adriamycin)
•Clozapine (sample brand names: Clozaril, FazaClo)
•Sulfasalazine (brand name: Azulfidine)
Vitamin deficiencies – Some vitamins help the body make neutrophils. Folic acid, vitamin B12, and copper are examples. You could have low levels of these vitamins if you are a strict vegetarian or have weight reduction surgery and do not take supplements.
Immune system problems – The immune system is the body's infection-fighting system. Normally, the immune system kills germs. But sometimes, it attacks healthy cells, including white blood cells. This condition is known as "autoimmune neutropenia." It can happen with certain diseases like lupus or rheumatoid arthritis.
People from certain ethnic groups can have a slightly low neutrophil count, but they have no health problems from this condition. This is called "benign ethnic neutropenia."
Problems related to the bone marrow – Some conditions that involve the blood cells made in the bone marrow can cause neutropenia. Examples include certain inherited conditions, myelodysplastic syndrome (MDS), and certain types of leukemia or anemia.
Is there a test for neutropenia? — Yes. A blood test called a "complete blood count with white blood cell differential" looks at all the different types of white blood cells in your blood. It can show if you have neutropenia. Doctors also call this test a "CBC with diff." The important number is the number of neutrophils, or "neutrophil count," not the percentage.
Your doctor or nurse will decide if you need other tests based on your age, other symptoms, and individual situation. Here are some of the things doctors use to find the cause of neutropenia:
CBC results – Sometimes there are other clues from the CBC, such as the sizes or numbers of other blood cells.
Other lab tests – Sometimes other lab tests may be helpful, such as the level of vitamin B12 or tests for lupus.
Bone marrow biopsy – For this, a doctor will take a very small sample of the bone marrow from your hip bone. Then another doctor will look at the cells under a microscope. Only some people need this test. Your doctor is most likely to do this test if you have a very low neutrophil count or other abnormal blood cells.
How is neutropenia treated? — The treatment depends on what caused you to get neutropenia.
If your neutropenia was caused by a medicine you took, your doctor might have you stop taking the medicine. He or she might be able to switch you to a different medicine that is less likely to cause the problem.
Your doctor might also prescribe drugs that help your bone marrow make white blood cells. These drugs are given in a shot and include:
Filgrastim (brand name: Neupogen)
Sargramostim (brand name: Leukine)
Your doctor will tell you how concerned you need to be about serious infections. If you are at risk for serious infections, you'll need to get to a doctor or hospital right away if you have a fever or other symptoms of infection. For people who have neutropenia from chemotherapy and get a fever, it is very important to get to the hospital right away to be treated for infection.
Am I at increased risk for blood cancer? — Despite some information that is out there, the answer for most people is no. Rarely, people who were born with some forms of severe neutropenia have a very slightly increased risk of a type of blood cancer called "acute myeloid leukemia." But this risk is small and applies only to a small group of people who are born with neutropenia, not people who get it later in life.
All topics are updated as new evidence becomes available and our peer review process is complete.
This topic retrieved from UpToDate on: Mar 30, 2020.
Topic 82906 Version 9.0
Release: 28.2.2 - C28.105
© 2020 UpToDate, Inc. and/or its affiliates. All rights reserved.