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Treatment of Newly Diagnosed HLH and Refractory Disease

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Histiocytic Disorders

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Abstract

To diagnose and treat a patient with hemophagocytic lymphohistiocytosis (HLH) is a challenge. Nevertheless, since the clinical course may be rapidly fatal and since the presentation may be variable, in particular for secondary HLH, it is important for clinicians in many medical fields to be aware of HLH and its treatment. Here, we review the treatment of patients with newly diagnosed HLH including therapy with corticosteroids, etoposide, cyclosporine A, and intravenous immunoglobulin (used in the HLH-94 and HLH-2004 protocols) as well as T-cell antibody-directed regimens with antithymocyte globulin (ATG) and alemtuzumab. We also report on anti-infectious and supportive therapy, prognostic factors, as well as the challenging treatment of secondary HLH, including infection-associated HLH, malignancy-associated HLH, and rheuma-/autoimmune-associated HLH. Finally, a literature review on salvage therapy for patients that fail to respond to standard HLH treatment is presented, including treatments with ATG, anakinra, and alemtuzumab.

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Abbreviations

ATG:

Antithymocyte globulin

CSA:

Cyclosporine A

DEP:

Doxorubicin, etoposide, and methylprednisolone

EBV:

Epstein-Barr virus

FHL:

Familial hemophagocytic lymphohistiocytosis

HLH:

Hemophagocytic lymphohistiocytosis

IFN:

Interferon

IL:

Interleukin

IVIG:

Intravenous immunoglobulin

MAS:

Macrophage activation syndrome

SCT:

Hematopoietic stem cell transplantation

sHLH:

Secondary HLH

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Author information

Authors and Affiliations

  1. 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, Stockholm, Sweden

    Jan-Inge Henter MD, PhD & Tatiana von Bahr Greenwood MD

  2. Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Rebecca A Marsh MD

Authors
  1. Jan-Inge Henter MD, PhD
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  2. Rebecca A Marsh MD
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  3. Tatiana von Bahr Greenwood MD
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Corresponding author

Correspondence to Jan-Inge Henter MD, PhD .

Editor information

Editors and Affiliations

  1. University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada

    Oussama Abla

  2. Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Hamburg, Germany

    Gritta Janka

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Henter, JI., Marsh, R.A., von Bahr Greenwood, T. (2018). Treatment of Newly Diagnosed HLH and Refractory Disease. In: Abla, O., Janka, G. (eds) Histiocytic Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-59632-7_14

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  • DOI: https://doi.org/10.1007/978-3-319-59632-7_14

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  • Online ISBN: 978-3-319-59632-7

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