Pathogenesis of hemophagocytic syndrome (HPS).

Abstract:

:Hemophagocytic syndrome (HPS) is a clinicopathologic entity characterized by increased proliferation and activation of benign macrophages with hemophagocytosis throughout the reticuloendothelial system. Uncontrolled T-lymphocyte activation is responsible for increased T(H)1 cytokines secretion such as IFN-gamma, IL-12 and IL-18 that promotes macrophage activation. Genetic defects specific for cytotoxic T lymphocytes (CTL) and natural killer (NK) cells have been identified in patients with primary HPS that are responsible for altered cell death and apoptosis induction or target killing. HPS may be secondary to malignancy, infection or autoimmune disease, and mechanisms involved are poorly understood. However, in adult-onset Still's disease, juvenile chronic arthritis and probably systemic lupus erythematosus, IL-18 might play a role in initiating macrophage activation.

journal_name

Autoimmun Rev

journal_title

Autoimmunity reviews

authors

Larroche C,Mouthon L

doi

10.1016/S1568-9972(03)00091-0

keywords:

subject

Has Abstract

pub_date

2004-02-01 00:00:00

pages

69-75

issue

2

eissn

1568-9972

issn

1873-0183

pii

S1568997203000910

journal_volume

3

pub_type

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