title: Acute myeloid leukemia following etoposide therapy for EBV-associated hemophagocytic lymphohistiocytosis: a case report and a brief review of the literature.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder characterized by prolonged fever, cytopenia, hepatosplenomegaly, rash, icterus, and other neurological symptoms. Successful treatment of HLH by etoposide has improved outcomes for children with HLH. However, the development of treatment-related acute myeloid leukemia (t-AML) after the usage of this drug is a concern.
  
  
  
  We report a case of acquired EBV-triggered HLH with progression to t-AML following etoposide therapy with cytogenetic abnormality for t (11; 19) (q23; p13) resulting in MLL gene fusion. The development of t-AML was detected 23 months after HLH diagnosis.
  
  
  
  Although the successful treatment of HLH by etoposide has improved outcomes for children with HLH, t-AML is a serious complication of topoisomerase II inhibitor therapy and is characterized by the presence of gene rearrangement. This study suggests that HLH patients undergoing therapy with HLH-2004 protocol need monitoring for future malignancy, especially in the initial 2 to 3 years.
authors.affiliation: Department of Pediatric Hematology, Affiliated Hospital of Qingdao University, 16 Jiangsu Road, Qingdao, Shandong, 266003, People's Republic of China.
authors.forename: Hua
authors.initials: H
authors.lastname: Pan
authors.affiliation: Department of Pediatrics, Qingdao Children's Hospital, 6 Tong An Road, Qingdao, Shandong, 266003, People's Republic of China.
authors.forename: Dong-Ning
authors.initials: DN
authors.lastname: Feng
authors.affiliation: Department of Pediatric Hematology, Affiliated Hospital of Qingdao University, 16 Jiangsu Road, Qingdao, Shandong, 266003, People's Republic of China.
authors.forename: Liang
authors.initials: L
authors.lastname: Song
authors.affiliation: Department of Pediatric Hematology, Affiliated Hospital of Qingdao University, 16 Jiangsu Road, Qingdao, Shandong, 266003, People's Republic of China. sunlr@vip.sina.com.
authors.forename: Li-Rong
authors.initials: LR
authors.lastname: Sun
mesh_terms: D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005047:Etoposide / Q000009:adverse effects* / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007223:Infant; D015470:Leukemia, Myeloid, Acute / Q000139:chemically induced* / Q000175:diagnosis; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000188:drug therapy* / Q000821:virology; D059005:Topoisomerase II Inhibitors / Q000009:adverse effects* / Q000627:therapeutic use
chemical_list: D059005:Topoisomerase II Inhibitors; D005047:Etoposide
keywords: Etoposide; Hemophagocytosis; Secondary AML