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Diseases

Genetic and Rare Diseases Information Center (GARD)

Cyclic neutropenia


Other Names for this Disease
  • Neutropenia cyclic
  • Cyclic hematopoiesis
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Treatment

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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.[1]

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.[1]

Common side effects of G-CSF include bone pain and headache, splenomegaly, and osteoporosis. Vasculitis, rashes, arthralgias, and glomerulonephritis have been infrequently reported.[1]

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.[1]

Last updated: 8/6/2010

References
  1. Dale DC. ELANE-Related Neutropenia. GeneReviews. 2009; http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=cyclic-n. Accessed 8/6/2010.


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Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions.
Other Names for this Disease
  • Neutropenia cyclic
  • Cyclic hematopoiesis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.