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Histiocytic Disorders pp 265–274Cite as

Hematopoietic Cell Transplantation and Novel Therapies in Hemophagocytic Lymphohistiocytosis

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Abstract

Hematopoietic cell transplantation (HCT) is currently the only curative therapy for primary HLH. While HCT carries substantial risks for patients with HLH, HCT is essential for preventing future episodes of fatal HLH in patients with genetic predisposition to HLH. Survival with HCT has increased significantly in recent years with the adoption of reduced intensity conditioning (RIC) regimens. However, RIC treatment protocols carry their own distinct issues including the frequent development of mixed chimerism. Patients with HLH are also challenging due to the potential for recurrence of HLH post HCT. Novel therapies, including interferon neutralization and genetic therapies, may facilitate initial and definitive therapy for future patients with HLH.

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Abbreviations

CNS:

Central nervous system

GVHD:

Graft-versus-host disease

HCT:

Hematopoietic cell transplantation

HLH:

Hemophagocytic lymphohistiocytosis

MAC:

Myeloablative conditioning

RIC:

Reduced intensity conditioning

UCB:

Umbilical cord blood

VOD:

Veno-occlusive disease

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Author information

Authors and Affiliations

  1. Clinical Research Division, Fred Hutchinson Cancer Research Center, Department of Pediatrics, University of Washington, Seattle, WA, USA

    K. Scott Baker MD, MS

  2. Divisions of Immunobiology, and Bone Marrow Transplantation and Immune Deficiency, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA

    Michael B. Jordan MD

Authors
  1. K. Scott Baker MD, MS
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  2. Michael B. Jordan MD
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Corresponding author

Correspondence to Michael B. Jordan MD .

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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Scott Baker, K., Jordan, M.B. (2018). Hematopoietic Cell Transplantation and Novel Therapies in Hemophagocytic Lymphohistiocytosis. In: Abla, O., Janka, G. (eds) Histiocytic Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-59632-7_15

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  • DOI: https://doi.org/10.1007/978-3-319-59632-7_15

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-59631-0

  • Online ISBN: 978-3-319-59632-7

  • eBook Packages: MedicineMedicine (R0)

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