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Difference between revisions of "Hemophagocytic syndrome"
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Hemophagocytic syndrome (view source)
Revision as of 18:26, 8 February 2011
, 18:26, 8 February 2011→Classification: more in genetic type
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==Classification== | ==Classification== | ||
Classified by etiology:<ref name=pmid20383897>{{cite journal |author=Gupta S, Weitzman S |title=Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy |journal=Expert Rev Clin Immunol |volume=6 |issue=1 |pages=137–54 |year=2010 |month=January |pmid=20383897 |doi= |url=}}</ref> | Classified by etiology:<ref name=pmid20383897>{{cite journal |author=Gupta S, Weitzman S |title=Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy |journal=Expert Rev Clin Immunol |volume=6 |issue=1 |pages=137–54 |year=2010 |month=January |pmid=20383897 |doi= |url=}}</ref> | ||
#Primary, i.e. inherited. | #Primary, i.e. inherited:<ref name=pmid21152410>{{cite journal |author=Nagai K, Yamamoto K, Fujiwara H, ''et al.'' |title=Subtypes of familial hemophagocytic lymphohistiocytosis in Japan based on genetic and functional analyses of cytotoxic T lymphocytes |journal=PLoS ONE |volume=5 |issue=11 |pages=e14173 |year=2010 |pmid=21152410 |pmc=2994802 |doi=10.1371/journal.pone.0014173 |url=}}</ref> | ||
#*Etiology: dysfunction of cytotoxic T lymphocytes with macrophage activating cytokines. | |||
#*Autosomal recessive. | |||
#*Two genes identified: | |||
#**FHL1.<ref>{{OMIM|603552}}</ref> | |||
#**FHL2.<ref>{{OMIM|603553}}</ref> | |||
#Secondary: | #Secondary: | ||
#*Infection, e.g. [[EBV]], [[HIV]]. | #*Infection, e.g. [[EBV]], [[HIV]]. | ||