Etoposide-based treatment of adult HLH is associated with high biochemical response but poor survival outcomes

Matthew C Nicholson; Leonie Naeije; Anna R Hayden; Andre Mattman; David Dix; Luke Y C Chen

doi:10.1002/jha2.57

Etoposide-based treatment of adult HLH is associated with high biochemical response but poor survival outcomes

EJHaem. 2020 Jul 14;1(1):277-280. doi: 10.1002/jha2.57. eCollection 2020 Jul.

Authors

Matthew C Nicholson¹, Leonie Naeije², Anna R Hayden¹, Andre Mattman³, David Dix², Luke Y C Chen^{1

4}

Affiliations

¹ Division of Hematology, Department of Medicine University of British Columbia Vancouver BC Canada.
² Division of Hematology, Oncology and Bone Marrow Transplant, Department of Paediatrics University of British Columbia Vancouver BC Canada.
³ Department of Pathology and Laboratory Medicine University of British Columbia Vancouver Canada.
⁴ Centre for Health Education Scholarship University of British Columbia Vancouver BC Canada.

PMID: 35847699
PMCID: PMC9175702
DOI: 10.1002/jha2.57

Abstract

Etoposide-based treatment is the standard of care for adult HLH in many centers, yet there remains a paucity of data regarding treatment outcomes. We conducted a retrospective study of 23 adults treated with etoposide-based therapy compared to 10 pediatric HLH cases at a single center. At diagnosis, the median serum ferritin was 20,071 µg/L and 937 µg/L in adults and children, respectively; median sIL-2r was 14,524 U/mL and 4,478 U/mL. Biochemical response to treatment was high, with 21/23 adults achieving >75% reduction in serum ferritin, but one year survival was only 7/21 compared to 7/10 in pediatric cases.

Keywords: HLH; etoposide; ferritin; hemophagocytic; soluble interleukin‐2 receptor.