SCN (severe congenital neutropenia) definition - medical term
An condition
characterized by a lack of neutrophils, a type of white blood cell
that is important in fighting infection. It is usually, but not
always, hereditary Children with severe congenital neutropenia (SCN)
suffer frequent infections from bacteria, which in the past led to
death in three-quarters of cases before three years of age. Children
with SCN have no special problems with viral or fungal infections,
but they do have an increased risk of developing acute myelogenous
leukemia or myelodysplasia, a bone marrow disorder. Aside from
agranulocytosis, the bone marrow and blood show a number of other
abnormalities, and the gamma globulin level in blood is low.
Hereditary SCN is an autosomal recessive disorder, so both parents
must carry an SCN gene to transmit the disease. Each child of two
parents with the SCN gene has a 25 percent risk of receiving both SCN
genes and therefore the disease.
Treatment with recombinant human
granulocyte colony-stimulating factor (GCSF) elevates the granulocyte
counts, helps resolve preexisting infections, diminishes the number
of new infections and results in significant improvements in survival
and quality of life. Some patients have developed leukemia or
myelodysplastic syndrome following treatment with GCSF, however.
Also known as Kostmann's disease or syndrome, infantile genetic
agranulocytosis, genetic infantile agranulocytosis.
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