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Hemophagocytic Lymphohistiocytosis Associated with Malignancies and with Epstein-Barr Virus

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) represents an often life-threatening syndrome associated with the uncontrolled activation of immune cells and the overproduction of pro-inflammatory cytokines. HLH can occur as the result of several underlying conditions in which there is excess stimulation of the immune system, in combination with inadequate downregulation of the immune response. Among the stimuli commonly associated with HLH are underlying malignancies, most often lymphomas, and certain viruses, including Epstein-Barr virus (EBV), a member of the herpesvirus family. In recent years, increased awareness has led to improved recognition as well as research into these as yet poorly understood disorders. We discuss current insights into the clinical manifestations, prognostic features, biology, and treatment approaches for malignancy- and EBV-associated HLH.

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Abbreviations

CAEBV:

Chronic active EBV infection

CMV:

Cytomegalovirus

CTL:

Cytotoxic T cells

EBNA:

Epstein-Barr nuclear antigen

EBV:

Epstein-Barr virus

FHL:

Familial HLH

FIM:

Fulminant mononucleosis

HIV:

Human immune deficiency virus

HLH:

Hemophagocytic lymphohistiocytosis

HSCT:

Hematopoietic stem cell transplantation

IFN:

Interferon

IM:

Infectious mononucleosis

ITK:

Inducible T cell kinase

MAC:

Myeloablative conditioning

MAGT1:

Magnesium transporter 1

MRI:

Magnetic resonance imaging

NK:

Natural killer

PBMNC:

Peripheral blood mononuclear cells

PTLD:

Post-transplantation lymphoproliferative disorder

RIC:

Reduced intensity conditioning

sCD25:

Soluble IL2 receptor

TBI:

Total body irradiation

TCR:

T cell receptor

TGF:

Transforming growth factor

UCBT:

Unrelated donor cord blood transplantation

VCA:

Virus capsid antigen

XLP:

X-linked lymphoproliferative disease

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Authors and Affiliations

  1. Department of Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Hamburg, Germany

    Kai Lehmberg MD

  2. Department of Pediatrics, Ehime University Graduate School of Medicine, Toon, Ehime, Japan

    Fumihiro Ochi MD & Eiichi Ishii MD

  3. Department of Oncology, St. Jude Children’s Research Hospital, 62 Danny Thomas Place, Memphis, TN, USA

    Kim E. Nichols MD

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  1. Kai Lehmberg MD
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  2. Fumihiro Ochi MD
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  3. Kim E. Nichols MD
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  4. Eiichi Ishii MD
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Correspondence to Kai Lehmberg MD .

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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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Lehmberg, K., Ochi, F., Nichols, K.E., Ishii, E. (2018). Hemophagocytic Lymphohistiocytosis Associated with Malignancies and with Epstein-Barr Virus. In: Abla, O., Janka, G. (eds) Histiocytic Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-59632-7_12

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