Cyclic Neutropenia

Overview

What is cyclic neutropenia (CyN)?

Neutropenia refers to lower-than-normal levels of neutrophils in the blood. A neutrophil (also known as a granulocyte or polymorphonuclear leukocyte) is a type of white blood cell produced in the bone marrow. White blood cells in general, and neutrophils in particular, are important for infection control in the body. A shortage of neutrophils render a person susceptible to infection.

Cyclic neutropenia (CyN) is not present all of the time. The number of circulating neutrophils decreases and normalizes on a regular basis. It is also called periodic neutropenia or cyclic hematopoiesis.

The periods of neutropenia typically last 3 to 5 days. The periods return every 14-35 days, often around 21 days.

How common is cyclic neutropenia (CyN)?

CyN is rare. There are an estimated 1-2 cases per 1 million people worldwide. The figure is estimated at 0.5 to 1 per 1 million people in the US.

Who is affected by cyclic neutropenia (CyN)?

The incidence is evenly split between male and female.

Symptoms and Causes

What causes cyclic neutropenia (CyN)?

CyN can occur later in life or be congenital (present from birth), but generally presents becomes evident right after birth. CyN is caused by a change in the genetic instructions (a mutation) to make an enzyme for fighting infection. Neutropenia symptoms result when the bone marrow does not produce enough neutrophils, when neutrophils die early, or when these cells do not perform correctly.

What are the symptoms of cyclic neutropenia (CyN)?

  • Fatigue
  • Fever
  • Sores in the mouth
  • Recurring gum problems and loose teeth
  • Sore throat
  • Skin infections
  • Lung infections
  • Digestive system infections
  • An overall feeling of not being well

Diagnosis and Tests

How is cyclic neutropenia (CyN) diagnosed?

Having an accurate family history is important in diagnosing CyN because it can be an inherited condition. Your doctor might order genetic tests to find any gene mutations. He or she may also order blood tests to measure the level of neutrophils two to three times per week for about six weeks.

Management and Treatment

What are the treatments for cyclic neutropenia (CyN)?

Treatments for CyN include:

  • Antibiotics for infections
  • Granulocyte colony-stimulating factor (G-CSF), filgrastim (Neupogen®)
  • Bone marrow transplant. This is rarely needed, but could be done if a person CyN does not respond to available treatments ordevelops cancer.

What are the complications/side effects of the treatments of cyclic neutropenia (CyN)?

Any person might be allergic to any type of medication. Signs of allergy include skin rashes or swelling, breathing difficulties and low blood pressure.

Antibiotics usually have some minor side effects, like diarrhea or yeast infections. Filgrastim side effects may include:

If you are taking filgrastim and have any type of problem, including pain in the upper left abdomen and/or shoulder, you should get immediate medical attention.

Prevention

How can you prevent cyclic neutropenia (CyN)?

CyN cannot be prevented. If there is a family history, you might want to speak with a genetic counselor for advice.

Outlook / Prognosis

What is the prognosis/outlook for patients who have CyN?

The outlook is often good, but some types of genetic mutations have been linked to a stronger risk of developing leukemia or other blood-related cancers.

Living With

When should I call the doctor if I have CyN/my child has CyN?

You should call your healthcare provider at once if you think that an infection is developing. The signs include:

  • Pain
  • Fever
  • Swelling or redness

You should take care to avoid infection where you can by:

  • Washing hands frequently
  • Avoiding getting injured when possible
  • Avoiding sick people or large groups of people in general
  • Getting recommended vaccinations
  • Avoiding touching your face, eyes and nose after touching anything
  • Practice good dental hygiene to help prevent periodontal disease.

Last reviewed by a Cleveland Clinic medical professional on 08/12/2019.

References

  • Genetic and Rare Diseases Information Center. Accessed 8/14/2019.Cyclic neutropenia. (https://rarediseases.info.nih.gov/diseases/6229/cyclic-neutropenia)
  • Genetics Home Reference. Accessed 8/14/2019.Cyclic neutropenia. (https://ghr.nlm.nih.gov/condition/cyclic-neutropenia)
  • National Organization for Rare Disorders. Accessed 8/14/2019.Cyclic neutropenia. (https://rarediseases.org/rare-diseases/cyclic-neutropenia/)
  • Dale D, Bolyard AA, Lueng J, Tran E, Marrero, TM, Newburger. . Blood 2017 130-2275. Accessed 8/14/2019.Cyclic neutropenia, congenital and idiopathic neutropenia (http://www.bloodjournal.org/content/130/Suppl_1/2275)
  • Patil VH, Hugar SM, Balikai G, Patil S. Int J Appl Basic Med Res. 2016;6(4):293-296. Accessed 8/14/2019. Severe congenital cyclic neutropenia: A case report. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5108111/)
  • Makaryan V, Zeidler C, Bolyard AA, et al. T. Curr Opin Hematol. 2015;22(1):3-11 Accessed 8/14/2019.he diversity of mutations and clinical outcomes for ELANE-associated neutropenia (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4380169/)

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