<unk>  <unk> , also often known as Kostmann syndrome or disease, is a group of rare disorders that affect myelopoiesis, causing a<unk>  form of<unk> , usually without other physical malformations.<unk>  manifests in infancy with life-threatening bacterial infections. Most cases of<unk>  respond to treatment with granulocyte colony-stimulating factor (filgrastim), which increases the neutrophil count and decreases the<unk> ty and frequency of infections. Although this treatment has significantly improved survival, people with<unk>  are at risk of long-term complications such as hematopoietic clonal disorders (myelodysplastic syndrome, acute myeloid leukemia). Kostmann disease <unk> 3), the initial subtype recognized, was clinically described in 1956. This type has an autosomal recessive inheritance pattern, whereas the most common subtype,<unk> 1, shows autosomal dominant inheritance.
Severe congenital neutropenia
Autoimmune neutropenia
Bare lymphocyte syndrome
Diffuse infiltrative lymphocytosis syndrome
Adult-onset immunodeficiency syndrome
Refractory cytopenia of childhood
Leukocyte adhesion deficiency
Myelodysplastic syndrome