Recommendations for the Use of Etoposide-Based Therapy and Bone Marrow Transplantation for the Treatment of HLH: Consensus Statements by the HLH Steering Committee of the Histiocyte Society.

Stephan Ehl, Itziar Astigarraga, Tatiana von Bahr Greenwood, Melissa Hines, AnnaCarin Horne, Eiichi Ishii, Gritta Janka, Michael B Jordan, Paul La Rosée, Kai Lehmberg, Rafal Machowicz, Kim E Nichols, Elena Sieni, Zhao Wang, Jan-Inge Henter

Journal: The journal of allergy and clinical immunology. In practice 2019;6(5):1508-1517

PMID: 30201097

Full Text on Elsevier Science View on PubMed

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome requiring aggressive immunosuppressive therapy. Following 2 large international studies mainly targeting pediatric patients with familial disease and patients without underlying chronic or malignant disease, the HLH-94 protocol is recommended as the standard of care when using etoposide-based therapy by the Histiocyte Society. However, in clinical practice, etoposide-based therapy has been widely used beyond the study inclusion criteria, including older patients and patients with underlying diseases (secondary HLH). Many questions remain around these extended indications and published reports do not address several practical issues. To tackle these concerns, the HLH Steering Committee of the Histiocyte Society decided to issue guidance for use of the HLH-94 protocol. The group convened in a structured consensus finding process to define recommendations that are based largely on expert opinion backed up by available data from the literature. The recommendations address all main elements of HLH-94 including corticosteroids, cyclosporin, etoposide, intrathecal therapy, and hematopoietic stem cell transplantation (HSCT) and consider various forms of HLH and all age groups. Aspects covered include indications, applications, dosing, side effects, duration of therapy, salvage therapy, and HSCT. These recommendations aim to provide a framework to guide treatment decisions in this severe disease.

Address: Center for Chronic Immunodeficiency, Faculty of Medicine, University of Freiburg, Freiburg, Germany; Center for Pediatrics and Adolescent Medicine, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany. Electronic address: [email protected].; Servicio de Pediatria, BioCruces Health Research Institute, Hospital Universitario Cruces, University of the Basque Country UPV/EHU, Barakaldo, Spain.; Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institutet, and Theme of Children's and Women's Health, Karolinska University Hospital Solna, Stockholm, Sweden.; Division of Critical Care Medicine, St. Jude Children's Research Hospital, Memphis, Tenn.; Department of Pediatrics, Ehime University Graduate School of Medicine, Ehime, Japan.; Clinic of Pediatric Hematology and Oncology, University Medical Center Eppendorf, Hamburg, Germany.; Divisions of Immunobiology and Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.; Klinik für Innere Medizin II, Schwarzwald-Baar-Klinikum, Villingen-Schwenningen, Germany.; Clinic of Pediatric Hematology and Oncology, Division of Pediatric Stem Cell Transplantation and Immunology, University Medical Center Eppendorf, Hamburg, Germany.; Department of Hematology, Oncology and Internal Diseases, Medical University of Warsaw, Warsaw, Poland.; Division of Cancer Predisposition, St. Jude Children's Research Hospital, Memphis, Tenn.; Department of Pediatric Hematology Oncology, Azienda Ospedaliero Universitaria A. Meyer Children Hospital, Firenze, Italy.; Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.; Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institutet, and Theme of Children's and Women's Health, Karolinska University Hospital Solna, Stockholm, Sweden. Electronic address: [email protected].

DOI: 10.1016/j.jaip.2018.05.031

Link outs

Free resources

Medical:

Other Literature Sources:

https://scite.ai/reports/30201097

Subscription / membership required

Full Text Sources:

MeSH Terms: Antineoplastic Agents, Phytogenic, Bone Marrow Transplantation, Consensus, Cytotoxicity, Immunologic, Etoposide, Health Planning Guidelines, Histiocytes, Humans, Lymphocyte Activation, Lymphocytes, Lymphohistiocytosis, Hemophagocytic, Societies, Medical