Home > Journals > Minerva Medica > Past Issues > Minerva Medica 2003 February;94(1) > Minerva Medica 2003 February;94(1):19-28

CURRENT ISSUE
 

ARTICLE TOOLS

Reprints

MINERVA MEDICA

A Journal on Internal Medicine


Indexed/Abstracted in: Current Contents/Clinical Medicine, EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,878


eTOC

 

REVIEWS  


Minerva Medica 2003 February;94(1):19-28

Copyright © 2003 EDIZIONI MINERVA MEDICA

language: Italian

The hemophagocytic syndrome (macrophage activation syndrome)

Fietta P., Manganelli P.


PDF  


The hemophagocytic syndrome (HPS) is an uncommon, often misdiagnosed life-threatening disorder of immune regulation, characterized by a widespread proliferation and multisystemic infiltration of non-malignant histiocytes that undergo uncontrolled hemophagocytosis in bone marrow and/or reticulo-endothelial system. The HPS immune dysfunction consists in a low or absent cytotoxic T and natural killercell activity and in hyperactivation of T lymphocytes and macrophages, with consequent proinflammatory cytokine storm. Clinically, HPS is characterized by high fever, lymphadenopathies, hepato-splenomegaly, liver dysfunction, (pan)cytopenia, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, as well as coagulopathy and neurological manifestations in many cases. A hereditary/primary variant (familial hemophagocytic lymphohistiocytosis) and an acquired/secondary form (reactive HPS) are known. This latter may occur in several disorders, including infections, immunodeficiency states, malignancies, lymphoproliferative and autoimmune diseases. Without treatment, HPS fatally has an unfavourable prognosis. Recently, outcome improvements have been reported, due to better therapeutic strategies. The clinical and pathological features of this syndrome are reviewed.

top of page

Publication History

Cite this article as

Corresponding author e-mail