Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study

Author:

Bergsten Elisabet12,Horne AnnaCarin12,Aricó Maurizio3,Astigarraga Itziar4,Egeler R. Maarten5,Filipovich Alexandra H.6,Ishii Eiichi7,Janka Gritta8,Ladisch Stephan9,Lehmberg Kai8,McClain Kenneth L.10,Minkov Milen11,Montgomery Scott121314,Nanduri Vasanta15,Rosso Diego16171819,Henter Jan-Inge12

Affiliation:

1. Childhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska Institute, Stockholm, Sweden;

2. Theme of Children’s and Women’s Health, Karolinska University Hospital, Stockholm, Sweden;

3. Direzione Generale, Azienda Sanitaria Provinciale, Ragusa, Italy;

4. Servicio de Pediatria, BioCruces Health Research Institute, Hospital Universitario Cruces, University of the Basque Country, Universidad del Pais Vasco–Euskal Herriko Unibertsitatea (UPV/EHU), Barakaldo, Spain;

5. Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, ON, Canada;

6. Division of Bone Marrow Transplant and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH;

7. Department of Pediatrics, Graduate School of Medicine, Ehime University, Ehime, Japan;

8. Pediatric Hematology and Oncology, University Medical Center Hamburg, Hamburg, Germany;

9. Center for Cancer and Immunology Research, Children's Research Institute, Children's National Medical Center, Washington, DC;

10. Texas Children's Cancer Center, Baylor College of Medicine, Houston, TX;

11. University Clinic of Pediatrics, St. Anna Children's Hospital, Medical University of Vienna, Vienna, Austria;

12. Clinical Epidemiology Unit, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden;

13. Clinical Epidemiology and Biostatistics, School of Medical Sciences, Örebro University, Örebro, Sweden;

14. Department of Epidemiology and Public Health, University College London, London, United Kingdom;

15. Department of Pediatrics, Watford General Hospital, Watford, United Kingdom;

16. Department of Pediatric Hematology and Oncology, Hospital de Niños Dr Pedro De Elizalde, Buenos Aires, Argentina;

17. Department of Pediatrics, Hospital de Clinicas Jose de San Martin, Buenos Aires, Argentina;

18. Instituto de Farmacología, Universidad de Buenos Aires, Buenos Aires, Argentina; and

19. Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Buenos Aires, Argentina

Abstract

Key Points Early introduction of cyclosporine did not improve HLH outcome in patients treated with the HLH-94 etoposide-dexamethasone backbone (P = .06). HLH-2004 may be improved by risk-group stratification, less therapy reduction weeks 7 to 8 for verified FHL patients, and earlier HSCT.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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