In vitro Funktionsuntersuchungen von G-CSF induzierten neutrophilen Granulozyten von Patienten mit schwerer angeborener Neutrophenie

: Roesler, J.; Emmendörffer, A.; Elsner, J.; Zeidler, C.; Lohmann-Matthes, M.-L.; Welte, K.

Abstract
Neutrophils from 5 patients suffering from severe congenital neutropenia (SCN) were investigated in vivo after induction of this cell type treatment with recombinant human granulocyte colony stimulating factor (rh G-CSF) in vivo. Some abnormal morphological features were seen, such as anomalies of nuclei of phagocytes. No limitations were found in the ability of neutrophils and monocytes to produce relative oxygen intermediates (ROI) following stimulation with phorbol-miristate-acetate (PMA) in the ability of neutrophils to phagocytose particles of zymosan A and to produce RIO simultaneously and in the ability to kill bacteria of the species staphylococcus aureus. However the specific migration of neutrophils in a gradient of FMLP under soft agar was decreased to approximately 50 per cent in all patients tested as compared to healthy controls. In addition, spontaneous motility was decreased in one patient. Nevertheless, the good clinical improvements of patients suffering from SCN afte r treatment with rh G-CSF appeared to be due to induction of neutrophils displaying overall good functional activities with respect to natural defense.