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 Revjournal_title
Autoimmunity reviewsauthors
Larroche C,Mouthon Ldoi
10.1016/S1568-9972(03)00091-0keywords:
subject
Has Abstractpub_date
2004-02-01 00:00:00pages
69-75issue
2eissn
1568-9972issn
1873-0183pii
S1568997203000910journal_volume
3pub_type
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