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Published in: BMC Pediatrics 1/2016

Open Access 01-12-2016 | Case report

Acute myeloid leukemia following etoposide therapy for EBV-associated hemophagocytic lymphohistiocytosis: a case report and a brief review of the literature

Authors: Hua Pan, Dong-ning Feng, Liang Song, Li-rong Sun

Published in: BMC Pediatrics | Issue 1/2016

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Abstract

Background

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.

Case presentation

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.

Conclusions

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.
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Metadata
Title
Acute myeloid leukemia following etoposide therapy for EBV-associated hemophagocytic lymphohistiocytosis: a case report and a brief review of the literature
Authors
Hua Pan
Dong-ning Feng
Liang Song
Li-rong Sun
Publication date
01-12-2016
Publisher
BioMed Central
Published in
BMC Pediatrics / Issue 1/2016
Electronic ISSN: 1471-2431
DOI
https://doi.org/10.1186/s12887-016-0649-z

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