What is severe congenital neutropenia (SCN)?
Severe congenital neutropenia is a bone marrow failure syndrome. Children with SCN have very low white blood counts (neutrophils) and are at risk for serious, life-threatening infections starting in infancy.
What are the signs and symptoms of SCN?
Children with SCN have normal physical examinations however they often experience infections in the first year of life. Children with SCN may have frequent fevers, thrush (yeast infections), skin boils or abscesses, and other life threatening infections.
How is SCN diagnosed?
Blood tests and a bone marrow examination will demonstrate low neutrophils. Genetic testing to identify mutation associated with SCN can be confirmatory in most cases. The most common genes affected in SCN include ELANE and HAX1.
How is SCN treated?
Treatment for severe congenital neutropenia in children usually begins with antibiotics to treat or prevent infection and recombinant human granulocyte colony-stimulating factor (GCSF). However, bone marrow transplant is the only curative treatment for this disease.
About treatment for severe congenital neutropenia at Children’s
Children’s Cancer and Blood Disorders program achieves outcomes that rank among the top national programs and cares for more than two-thirds of Minnesota children and adolescents with blood disorders. In the program, families coping with anemia have access to the newest and most promising treatments and receive care spearheaded and coordinated by a board-certified hematologist/oncologist.
If you are a family member looking for a Children’s hematologist or oncologist or wanting to schedule an appointment, call the outpatient clinic at Children’s – Minneapolis at 612-813-5940.
If you are a health professional looking for consultation or referral information, please call Children’s Physician Access at 1-866-755-2121 (toll-free).