Waterhouse-Friderichsen Syndrome with Excessive Hypercytokinemia

We report a case of Waterhouse-Friderichsen syndrome with excessive hypercytokinemia in a previously healthy 59-year-old man. The patient complained of a 20 hour history of fever and multiple pain. He was in shock on arrival and diffuse petechiae were observed on his trunk. The laboratory data evaluated metabolic acidosis (BE-16.9mmol/l), leukopenia (WBC 3, 900/μl) with a shift to the lef, obvious thrombocytopenia (1.8×104/μl), hypoglycemia (49mg/dl), and disseminated intravascular coagulation (DIC). Abdominal computerized tomography (CT) scan showed bilateral adrenal swelling and irregular shape of left gland. Immediately he developed adult respiratory distress syndrome (ARDS), then died 17.5 hours after arrival without recovery from shock. On microbiological investigation, group 12 (Danish classification) of penicillin sensitive Streptococcus pneumniae was detected in his blood. The serum data demonstrated excessive hypercytokinemia (interleukin [IL]-1β 1, 010pg/ml, IL-6 1.03×106pg/ml). An autopsy revealed massive hemorrhagic necrosis and fibrinous thrombosis in a half of bilateral adrenal cortex and DIC in multiple organs, while the spleen was intact. Neither focus of original infection nor what aggravated the infection was clear.