Article
Kostmann's Disease and HCLS1-Associated Protein X-1 (HAX1).
Journal of clinical immunology - 1 Feb 2017
Klein Christoph
Abstract excerpt
Severe congenital neutropenia (SCN), originally described by the Swedish pediatrician Rolf Kostmann, constitutes a heterogeneous disorder associated with a dramatic decrease of peripheral neutrophil granulocytes. Patients suffer from life-threatening bacterial infections unless treated by recombinant human granulocyte colony stimulating factor (G-CSF) or allogeneic hematopoietic stem cells. This review is focused...
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