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