2019 Volume 39 Issue 5 Pages 504-509
A 20-year-old woman with fever and general fatigue was transferred to our intensive care unit because of pancytopenia, liver and kidney dysfunction, and disseminated intravascular coagulation. She was diagnosed with hemophagocytic lymphohistiocytosis（HLH） according to the HLH 2004 guidelines. Virological tests on admission indicated a high serum Epstein-Barr virus（EBV）load, and she was diagnosed with EBV-associated HLH on the 4th day. Administration of high dose methylprednisolone, continuous hemodiafiltration（CHDF）and plasma exchange（PE）was initiated. Despite the treatment, her condition deteriorated and she was intubated and ventilated mechanically on the 7th day because of alveolar hemorrhage. After implementing etoposide in accordance with the HLH 2004 guidelines on the 8th day, her condition started to improve. She became free from mechanical ventilation and PE on the 13th day and from CHDF on the 14th day and recovered without subsequent sequelae. Prompt initiation of etoposide and cyclosporine was important for refractory adult EBV-associated HLH.
