Kostmann syndrome: Difference between revisions
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imported>Howard C. Berkowitz (New page: First described in 1956,<ref>{{citation | author = Kostmann R. Infantile genetic agranulocytosis. A new recessive lethal disease in man | jorunal = Acta Paediatr Scand | year = 1956 | ...) |
imported>Howard C. Berkowitz m (Kostmann Syndrome moved to Kostmann syndrome) |
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Revision as of 15:24, 30 July 2010
First described in 1956,[1] Kostmann syndrome, also called congenital neutropenia, is characterized as "is characterized by an arrest of the maturation of neutrophil precursors at the promyelocytic stage of differentiation and low levels of mature neutrophils in peripheral blood." It was generally lethal before treatment with granulocyte colony-stimulating factor was available, although some individuals were protected with antibiotics.[2]
References
- ↑ Kostmann R. Infantile genetic agranulocytosis. A new recessive lethal disease in man (1956), at 1-78
- ↑ Carlsson G et al. , "Neutrophil elastase and granulocyte colony-stimulating factor receptor mutation analyses and leukemia evolution in severe congenital neutropenia patients belonging to the original Kostmann family in northern Sweden", Haematologica 91 (5): 589