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HLH in Adults

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Histiocytic Disorders

Abstract

Hemophagocytic lymphohistiocytosis (HLH) in adolescent and adult patients (aHLH) is getting increasing attention due to an assumed rising incidence, a better pathophysiologic understanding, and a growing arsenal of therapeutic ammunition. For many years HLH used to be a domain of pediatrics and was almost totally neglected in adults. Nowadays, with raising awareness (although it is still severely underdiagnosed), more and more physicians face the challenges of diagnosis and treatment of aHLH. The aim of this chapter is to provide information on HLH, specifically in adults. The spectrum of underlying conditions, triggering factors, and genetic predisposition differs in age groups with enrichment of familial disease in children and enrichment of malignant disorders as underlying condition and/or trigger in adults. These differences plus differential tolerance of drugs due to age and comorbidity guide the recommended aHLH treatment approach. Simply copying the pediatric recommendations to adults may have deleterious consequences. Treatment of HLH requires individual tailoring of anti-inflammatory and immunosuppressive treatment components combined with disease-specific treatment such as chemotherapy in malignancy or antimicrobial drugs in infection-triggered HLH. The clinical overlap to sepsis with high risk of missed diagnosis on intensive care units will also be discussed.

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Abbreviations

aHLH:

adult hemophagocytic lymphohistiocytosis

AOSD:

adult-onset Still’s disease

CS:

corticosteroids

CsA:

cyclosporin A

CTL:

cytotoxic T lymphocyte

DEX:

dexamethasone

EBMT:

European Society of Blood and Marrow transplantation

EBV:

Epstein-Barr virus

FHL:

familial HLH

HLH:

hemophagocytic lymphohistiocytosis

LA-HLH:

lymphoma-associated HLH

MAS:

macrophage activation syndrome

MOF:

multiorgan failure

NK cell:

natural killer cell

PVIG:

polyvalent intravenous immunoglobulin

sCD25:

soluble interleukin-2 receptor

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Authors and Affiliations

  1. Klinik für Innere Medizin II, Klinikstr. 11, Schwarzwald-Baar-Kinikum, Villingen-Schwenningen, Germany

    Paul La Rosée MD

  2. Department of Hematology, Oncology and Internal Diseases, Medical University of Warsaw, ul. Banacha 1a, 02-097, Warsaw, Poland

    Rafal Machowicz MD, MSc

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  1. Paul La Rosée MD
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Correspondence to Paul La Rosée 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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La Rosée, P., Machowicz, R. (2018). HLH in Adults. In: Abla, O., Janka, G. (eds) Histiocytic Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-59632-7_16

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