Linked Life Data

14596017

Source:http://linkedlifedata.com/resource/pubmed/id/14596017

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
37
pubmed:dateCreated
2003-11-4
pubmed:abstractText
Reactive macrophage activation syndrome or haemophagocytic lymphohistiocytosis is a life-threatening immunoregulatory disorder characterized by the activation of the mononuclear phagocytic system with prominent haemophagocytosis particularly involving the bone marrow, reticuloendothelial system and central nervous system. It frequently occurs in children as a familial autosomal recessive disorder and it has been reportedly associated with infections, underlying hematopoetic malignancies or autoimmune disorders. It has been suggested that central to the pathogenesis of this disorder is uncontrolled T-cell activation which results in the excessive secretion of cytokines. The clinical and laboratory findings are dramatic, at presentation with persistent fever, hepatosplenomegaly, profound depression of three blood cell lines, elevated serum liver enzyme values, coagulopathy and abundant haemophagocytosis can be visualized in bone marrow. Most of the described clinical findings could be explained by the oversecretion of proinflammatory cytokines and the presence of histiocytic infiltrates. In general, immunosuppressive therapy, cytotoxic chemotherapy with immunomodulatory agents will induce remissions in a substantial percentage of patients, whereas others require allogenic bone marrow transplantation.
pubmed:language
hun
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Sep
pubmed:issn
0030-6002
pubmed:author
pubmed:issnType
Print
pubmed:day
14
pubmed:volume
144
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1803-8
pubmed:dateRevised
2009-10-21
pubmed:meshHeading
pubmed:year
2003
pubmed:articleTitle
[Reactive macrophage activation syndrome].
pubmed:affiliation
Debreceni Egyetem, Orvos- és Egészségtudományi Centrum Altalános Orvostudományi Kar, III. Belgyógyászati Klinika, Klinikai Immunológia Tanszék.
pubmed:publicationType
Journal Article, English Abstract, Review