Classification, Clinical Manifestations, and Diagnostics of HLH

  • J. Pachlopnik Schmid
  • Benjamin Volkmer
  • Stephan Ehl
Chapter

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune dysregulation disease. It can be subdivided into a primary, genetic form and a secondary form that complicates diverse infections, autoimmune or autoinflammatory disorders, and malignancies. Both forms, primary and secondary HLH, present with the same spectrum of non-specific symptoms, making accurate diagnosis and rapid treatment initiation challenging. A prompt diagnosis is crucial for appropriate therapeutic management. Clinical diagnosis of an HLH episode, followed by sequential immunological testing, and genetic diagnosis provide the basis for a rapid decision on hematopoietic stem cell transplantation indicated in patients with primary HLH.

Keywords

Classification Clinical manifestation Organ involvement Laboratory features Diagnosis Immunological testing Genetic analyses Hemophagocytic lymphohistiocytosis Macrophage activation syndrome 

Abbreviations

ADV

Adenovirus

CGD

Chronic granulomatous disease

CHS

Chediak-Higashi syndrome

CNS

Central nervous system

CSF

Cerebrospinal fluid

EBV

Epstein-Barr virus

FHL

Familial HLH

HHV

Human herpes virus

HLH

Hemophagocytic lymphohistiocytosis

HPS

Hermansky-Pudlak syndrome

HSCT

Hematopoietic stem cell transplantation

IFN

Interferon

MAS

Macrophage activation syndrome

MRI

Magnetic resonance imaging

NGS

Next-generation sequencing

PBMCs

Peripheral blood mononuclear cells

PCR

Polymerase chain reaction

PID

Primary immunodeficiency

SAP

Signaling lymphocytic activation molecule-associated protein

sJIA

Systemic juvenile idiopathic arthritis

SLE

Systemic lupus erythematosus

WES

Whole-exome sequencing

WGS

Whole-genome sequencing

XIAP

X-linked inhibitor of apoptosis

XLP

X-linked lymphoproliferative syndrome

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Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  • J. Pachlopnik Schmid
    • 1
  • Benjamin Volkmer
    • 1
  • Stephan Ehl
    • 2
  1. 1.Division of ImmunologyUniversity Children’s Hospital, University of ZurichZurichSwitzerland
  2. 2.Center for Chronic ImmunodeficiencyUniversity Medical Center Freiburg, University of FreiburgFreiburgGermany

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