A case of acute myeloid leukemia with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis successfully treated by cord blood transplantation

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory life-threatening syndrome. Epstein-Barr virus (EBV) infection is a common cause of HLH. Occasionally, HLH develops with malignancies such as lymphoma. Concurrent cases of acute myeloid leukemia (AML) and EBV-associated HLH (EBV-HLH) are very rare, and the treatment has not been established. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment for refractory HLH and high-risk AML. Consequently, HSCT would be a good treatment for cases of HLH and high-risk AML coexistence. A 66-year-old man was diagnosed with high-risk AML. After induction chemotherapy, he experienced prolonged pancytopenia, high fever, and elevated liver enzymes. A high EBV viral load was found, and he was diagnosed with EBV-HLH, with EBV infecting CD8+ T-cells. EBV-HLH was resolved by treatment with corticosteroids. Meanwhile, some hemophagocytosis remained after consolidation chemotherapy, and EBV-DNA in whole blood was positive. The patient received cord blood transplantation to treat both AML and EBV-HLH. His AML achieved complete remission, and the hemophagocytosis was in remission. However, his EBV viral load increased again 53 days after transplantation. Then, the EBV infection occurred in Bcells, and there was no relapse of hemophagocytosis. In conclusion, both AML and HLH were successfully treated by transplantation. This report highlights treatment strategies in specific cases of AML and EBV-HLH coexistence.