Recently, some of the mechanisms and consequences in the severe chronic neu
tropenias (e.g. the neutrophil elastase gene mutations and the risk to prog
ress to myelodysplasia and acute leukaemia) and in drug-induced agranulocyt
osis (e.g. the apoptosis-inducing ability of metabolites of clozapine) have
been elucidated, and new aspects of autoimmune and the large granular lymp
hocyte syndrome were described (e.g. aberrant elaboration of Fas-ligand cau
sing neutrophil apoptosis). Investigations of the mild to moderate chronic
neutropenias have shown the significance of interactions between the myeloi
d development and the immune network (e.g. relations to immunoglobulin aber
rations). Granulocyte-colony stimulation factor (G-CSF) is Widely used in p
atients with severe chronic neutropenia, however, its use in other conditio
ns is mostly based on anecdotal evidence. In addition, immune modulating re
gimens, such as metothrexate, ciclosporine and monoclonal antibodies, are i
ncreasingly employed for the autoimmune neutropenias.