Severe agranulocytosis in two patients with drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms

Abstract

Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DIHS/ DRESS) is a life-threatening adverse reaction characterized by skin rashes, fever, leukocytosis with eosinophilia and/or atypical lymphocytosis, lymph node enlargement, and liver and/or renal dysfunctions (1, 2). A wide variety of other involvements have also been reported, including limbic encephalitis, myocarditis, and gastrointestinal disease, developing during the course of the disease (3–5). It has been demonstrated that human herpesvirus 6 (HHV-6), Epstein-Barr virus (EBV) and cytomegalovirus (CMV) reactivate during the course of the disease and some organ involvements are associated with these herpesvirus reactivations (6, 7). Although haematological abnormalities, such as leukocytosis, eosinophilia, and atypical lymphocytosis, are characteristic symptoms of DIHS/DRESS, little attention has been paid to other haematological abnormalities, such as neutropaenia, agranulocytosis, and leukemoid reaction, detected in the course of the disease, even though these abnormalities could induce life-threatening events (8–12). We report here 2 patients who developed agranulocytosis in the course of DIHS/DRESS.