Severe congenital neutropenia
K Welte, C Zeidler, DC Dale - Seminars in hematology, 2006 - Elsevier
Severe congenital neutropenia (CN) includes a variety of hematologic disorders
characterized by severe neutropenia, with absolute neutrophil counts (ANC) below 0.5×
109/L, and associated with severe systemic bacterial infections from early infancy. One
subtype of CN, Kostmann syndrome, is an autosomal recessive disorder, characterized
histopathologically by early-stage maturation arrest of myeloid differentiation. CN with
similar clinical features occurs as an autosomal dominant disorder and many sporadic cases …
characterized by severe neutropenia, with absolute neutrophil counts (ANC) below 0.5×
109/L, and associated with severe systemic bacterial infections from early infancy. One
subtype of CN, Kostmann syndrome, is an autosomal recessive disorder, characterized
histopathologically by early-stage maturation arrest of myeloid differentiation. CN with
similar clinical features occurs as an autosomal dominant disorder and many sporadic cases …