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NEUTROPENIA

(KOSTMANN'S SYNDROME)
Neutropenia

WHAT YOU NEED TO KNOW:

What is neutropenia? Neutropenia is a condition that causes you to have a low number of neutrophils in your blood. Neutrophils are a type of white blood cell made in the bone marrow. They help your body fight infection and bacteria.

What increases my risk for neutropenia?

  • Family history or inherited genes

  • Medical treatments, such as chemotherapy or radiation therapy

  • Certain medicines, such as penicillin or aspirin

  • Infections, such as Hepatitis A or B, RSV, influenza A or B, cytomegalovirus, Epstein-Barr virus, and HIV

  • Autoimmune disorders, including hyperthyroidism, rheumatoid arthritis, and lupus

  • Bone marrow diseases, including aplastic anemia and acute leukemia

  • Lack of certain vitamins and minerals, such as B12, folate, and copper

What are the signs and symptoms of neutropenia? You may have no signs or symptoms, or you may have any of the following:

  • Fever

  • Bruises, splotchy red spots, or sores on your skin

  • Mouth sores

  • Sinus infections

  • Anal or rectal sores

  • Fast heartbeat

  • Cough or wheezing

How is neutropenia diagnosed? Your healthcare provider will examine you. He may ask about the medicine you take or about other health conditions you have. He may check for signs of infection. You may also need the following tests:

  • Blood tests, such as blood cultures, will show if your body is working properly. Tests will also show if you have an infection and if your neutropenia is moderate or severe.

  • Urine tests will show if you have an infection of your bladder or kidneys.

  • CT scan is also called a CAT scan. An x-ray machine uses a computer to take pictures of your lungs, abdomen, brain, or sinuses. The pictures may show infection, stroke, or a sinus infection. You may be given dye to help healthcare providers see the pictures better. Tell the healthcare provider if you have ever had an allergic reaction to contrast dye.

How is neutropenia treated? Treatment will depend on the cause of your neutropenia. Your healthcare provider will treat any infections you have. You may also need to change the foods you eat to make sure you get the right nutrition. You may need to stop taking medicines that can cause neutropenia. You may also receive growth factor to help stimulate your bone marrow to make more neutrophils.

What are the risks of neutropenia? Neutropenia may cause infections of your skin, mouth, and anal areas. It may cause a serious infection throughout your body, called sepsis. You may need to be hospitalized. If untreated, the infection may be life-threatening.

How can I prevent infections if I have neutropenia? Ask your healthcare provider about these and other precautions you may need to prevent an infection:

  • Wash your hands before you prepare or eat food, and after you use the bathroom.

  • Bathe daily. If you shave, use an electric razor to prevent nicks in your skin.

  • Use a soft-bristled toothbrush, and brush your teeth gently 2 times each day. Ask your healthcare provider if it is okay for you to gently floss daily.

  • Avoid crowds and anyone who may be sick.

  • Avoid contact with animal saliva, urine, or feces. Have someone clean your cat's litter box, fish tank, or pick up after your dog.

  • Wash raw fruits and vegetables thoroughly. Cook meats and eggs thoroughly.

  • Use stool softeners to help with constipation. Do not use suppositories or enemas. Constipation, suppositories, and enemas can cause a tear in your rectum. This allows germs to get in and can increase your risk for infection.

  • Ask your healthcare provider if you should get the flu vaccine every fall.

When should I contact my healthcare provider?

  • You have fever or chills.

  • You have a new cough.

  • You have a sore throat or a new mouth sore.

  • You have redness or swelling any place on your body.

  • You have pain in your abdomen or rectum.

  • You have burning or pain when you urinate.

  • You have diarrhea.

  • You are more tired or forgetful than usual.

  • You have questions or concerns about your condition or care.

When should I seek immediate care?

  • You have a fever of 100.4°F (38°C) for more than 1 hour.

  • You have a fever of 101°F (38.3°C) or higher once.

CARE AGREEMENT:

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.