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dc.contributor.authorUsmani, G. Naheed
dc.contributor.authorWoda, Bruce A.
dc.contributor.authorNewburger, Peter E.
dc.date2022-08-11T08:08:29.000
dc.date.accessioned2022-08-23T15:56:52Z
dc.date.available2022-08-23T15:56:52Z
dc.date.issued2013-06-01
dc.date.submitted2013-06-05
dc.identifier.citationBr J Haematol. 2013 Jun;161(5):609-22. doi: 10.1111/bjh.12293. Epub 2013 Apr 12. <a href="http://dx.doi.org/10.1111/bjh.12293">Link to article on publisher's site</a>
dc.identifier.issn0007-1048 (Linking)
dc.identifier.doi10.1111/bjh.12293
dc.identifier.pmid23577835
dc.identifier.urihttp://hdl.handle.net/20.500.14038/30065
dc.description.abstractHaemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory disorder resulting from immune dysfunction reflecting either primary immune deficiency or acquired failure of normal immune homeostasis. Familial HLH includes autosomal recessive and X-linked disorders characterized by uncontrolled activation of T cells and macrophages and overproduction of inflammatory cytokines, secondary to defects in genes encoding proteins involved in granule-dependent cytolytic pathways. In older children and adults, HLH is associated more often with infections, malignancies, autoimmune diseases, and acquired immune deficiencies. HLH, macrophage activation syndrome, sepsis, and systemic inflammatory response syndrome are different clinical entities that probably represent a common immunopathological state, termed cytokine storm. These conditions may be clinically indistinguishable; all include massive inflammatory response, elevated serum cytokine levels, multi-organ involvement, haemophagocytic macrophages, and often death. Tissues of haematopoietic and lymphoid function are directly involved; other organs are secondarily damaged by circulating cytokines and chemokines. Haemophagocytic disorders are now increasingly diagnosed in the context of severe inflammatory reactions to viruses, malignancies and systemic connective tissue diseases. Many of these cases may reflect underlying genetic predispositions to HLH. The detection of gene defects has contributed considerably to our understanding of HLH, but the mechanisms leading to acquired HLH have yet to be fully determined.
dc.language.isoen_US
dc.relation<a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&list_uids=23577835&dopt=Abstract">Link to Article in PubMed</a>
dc.relation.urlhttp://dx.doi.org/10.1111/bjh.12293
dc.subjectLymphohistiocytosis, Hemophagocytic
dc.subjectHemic and Lymphatic Diseases
dc.subjectPathology
dc.titleAdvances in understanding the pathogenesis of HLH
dc.typeJournal Article
dc.source.journaltitleBritish journal of haematology
dc.source.volume161
dc.source.issue5
dc.identifier.legacycoverpagehttps://escholarship.umassmed.edu/faculty_pubs/3
dc.identifier.contextkey4199933
html.description.abstract<p>Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory disorder resulting from immune dysfunction reflecting either primary immune deficiency or acquired failure of normal immune homeostasis. Familial HLH includes autosomal recessive and X-linked disorders characterized by uncontrolled activation of T cells and macrophages and overproduction of inflammatory cytokines, secondary to defects in genes encoding proteins involved in granule-dependent cytolytic pathways. In older children and adults, HLH is associated more often with infections, malignancies, autoimmune diseases, and acquired immune deficiencies. HLH, macrophage activation syndrome, sepsis, and systemic inflammatory response syndrome are different clinical entities that probably represent a common immunopathological state, termed cytokine storm. These conditions may be clinically indistinguishable; all include massive inflammatory response, elevated serum cytokine levels, multi-organ involvement, haemophagocytic macrophages, and often death. Tissues of haematopoietic and lymphoid function are directly involved; other organs are secondarily damaged by circulating cytokines and chemokines. Haemophagocytic disorders are now increasingly diagnosed in the context of severe inflammatory reactions to viruses, malignancies and systemic connective tissue diseases. Many of these cases may reflect underlying genetic predispositions to HLH. The detection of gene defects has contributed considerably to our understanding of HLH, but the mechanisms leading to acquired HLH have yet to be fully determined.</p>
dc.identifier.submissionpathfaculty_pubs/3
dc.contributor.departmentDepartment of Pathology
dc.contributor.departmentDepartment of Pediatrics
dc.source.pages609-22


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