Non chemotherapy drug-induced agranulocytosis is considered a potentially life-threatening idiosyncratic blood dyscrasia, thought to result from a partly elucidated immune and/or toxic damage on myelopoiesis, due to a multitude of drugs. Offending agents include clozapine, ticlopidine, antithyroid compounds, dipyrone, sulfasalzine, trimethropim/sulfomethoxazole, carmabazepine, and to a lesser extent, deferiprone (L1) and probably rituximab. Suspected drugs should be immediately stopped and, in symptomatic patients, once appropriate cultures have been obtained, broad-spectrum antibiotic treatment should be administered. Hematopoietic growth factors may be considered, specifically in patients with poor prognostic factors. Due to improved intensive care treatment and alertness of physicians the case fatality of the disorder has recently been decreased to 5%.