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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Cyclic neutropenia


Other Names for this Disease
  • Cyclic hematopoiesis
  • Neutropenia cyclic
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Your Question

My daughter has cyclic neutropenia. She experiences severe side effects when taking Neupogen injections (granulocyte colony-stimulating factor or G-CSF). Are there any dietary ways to control the symptoms of cyclic neutropenia?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is cyclic neutropenia?

Cyclic neutropenia is a rare blood disorder characterized by recurrent episodes of abnormally low levels of neutrophils (a type of white blood cell) in the body. Neutrophils are instrumental in fighting off infection by surrounding and destroying bacteria that enter the body. Symptoms of cyclic neutropenia may include fever, a general feeling of ill health, and/or sores (ulcers) of the mucous membranes of the mouth. Individuals with low levels of neutrophils (neutropenia) are highly susceptible to recurrent infections.[1] Cyclic neutropenia may be inherited or acquired. Some cases are present at birth and appear to occur randomly for no apparent reason (sporadic). Inherited cases appear to be transmitted in an autosomal dominant fashion and caused by mutations in the ELANE gene.[2]
Last updated: 10/18/2011

How might cyclic neutropenia be treated?

Conventional management includes prompt treatment of fevers and infections with antibiotics. Individuals with abdominal pain require careful evaluation for the potentially lethal complications of peritonitis and bacteremia.[3]

Treatment with granulocyte colony-stimulating factor (G-CSF), also called Neupogen, is effective in elevating blood neutrophil counts in cyclic neutropenia. G-CSF treatment ameliorates the symptoms and problems of infections in almost all affected individuals. In cyclic neutropenia, G-CSF shortens the periods of neutropenia as well as the length of the neutropenic cycle. Treatment is known to be effective at least as early as age six months to one year. Studies indicate that treatment is effective with no adverse effects on growth, development, or pregnancy outcome with follow-up to age 18 years.[3]

Common side effects of G-CSF include bone pain and headache, splenomegaly, and osteoporosis. Vasculitis, rashes, arthralgias, and glomerulonephritis have been infrequently reported.[3]

For affected individuals with a well-matched donor, hematopoietic stem cell transplantation (HSCT) may be the preferred treatment option. HSCT is the only alternative therapy for individuals with congenital neutropenia who are refractory to high-dose G-CSF or who undergo malignant transformation.[3]

Last updated: 8/6/2010

Where can I access more detailed information about the treatment of cyclic neutropenia?

Additional information about the treatment of cyclic neutropenia can be access through the following web resources.

GeneReviews
eMedicine
Last updated: 8/6/2010

Are there any dietary aways to control the symptoms of cyclic neutropenia?

While patients with cyclic neutropenia are advised to avoid fresh fruits, vegetables, and flowers during severe neutropenia in order to eliminate possible sources of infection, there do not appear to be any dietary ways to control the symptoms of cyclic neutropenia.[4]  
Last updated: 8/6/2010

References