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Macrophage Activation Syndrome

  • Esraa M. Eloseily
  • Randy Q. Cron
Chapter

Abstract

Hemophagocytic lymphohistiocytosis (HLH), or termed macrophage activation syndrome (MAS) when associated with rheumatic disorders, is a frequently fatal complication of infections, rheumatic disorders, and hematopoietic malignancies. Clinically, HLH/MAS is a life-threatening condition that is usually diagnosed among febrile hospitalized patients (children and adults) who commonly present with unremitting fever and a shock-like multiorgan dysfunction scenario. Laboratory studies reveal pancytopenia, elevated liver enzymes, elevated markers of inflammation (ESR, CRP), hyperferritinemia, and features of coagulopathy. In about 60% of cases, excess hemophagocytosis (macrophages/histiocytes engulfing other hematopoietic cell types) is noted on biopsy specimens from the bone marrow, liver, lymph nodes, and other organs. HLH/MAS has been hypothesized to occur when a threshold level of inflammation has been achieved, and genetic and environmental risk factors are believed to contribute to the hyperinflammatory state. A broad variety of infections, from viruses to fungi to bacteria, have been identified as triggers of HLH/MAS, either in isolation or in addition to an underlying inflammatory disease state. Certain infections, particularly by members of the herpesvirus family, are the most notorious triggers of HLH/MAS. Treatment for infection-triggered MAS requires therapy for both the underlying infection and dampening of the hyperactive immune response.

Keywords

Cytokine storm Epstein-Barr virus Hemophagocytic lymphohistiocytosis Interleukin-1 Macrophage activation syndrome 

Abbreviations

AIDS

Acquired immunodeficiency syndrome

APC

Antigen-presenting cell

CsA

Cyclosporine A

CTL

Cytotoxic T lymphocyte

CTLA-4

Cytotoxic T-lymphocyte-associated protein 4

DIC

Disseminated intravascular coagulopathy

EBV

Epstein-Barr virus

fHLH

Familial hemophagocytic lymphohistiocytosis

FSTL-1

Follistatin-like 1

G-CSF

Granulocyte colony-stimulating factor

GM-CSF

Granulocyte-macrophage colony-stimulating factor

HAV

Hepatitis A virus

HBV

Hepatitis B virus

HCV

Hepatitis C virus

HIV

Human immunodeficiency virus

HLH

Hemophagocytic lymphohistiocytosis

HS

HScore

HSCT

Hematopoietic stem cell transplant

ICU

Intensive care unit

IFN-γ

interferon-gamma

IL

Interleukin

IL-18BP

Interleukin-18-binding protein

IL-1Ra

Interleukin-1 receptor antagonist

IVIg

Intravenous immunoglobulin

MAS

Macrophage activation syndrome

NK cell

Natural killer cell

sCD163

Soluble haptoglobin receptor

sCD25

Soluble interleukin-2 receptor alpha chain

sHLH

Secondary hemophagocytic lymphohistiocytosis

sJIA

Systemic juvenile idiopathic arthritis

SLE

Systemic lupus erythematosus

Th1

T-helper 1

TLR

Toll-like receptor

TNF

Tumor necrosis factor

USA

United States of America

XLP

X-linked lymphoproliferative disease

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Assiut University Children’s HospitalAssiutEgypt
  2. 2.University of Alabama at BirminghamBirminghamUSA

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