Difference between revisions of "Hemophagocytic syndrome"
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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]]. | ||
Revision as of 18:26, 8 February 2011
Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis, is a rare condition often associated with viral infections.
Clinical
Features:[1]
- Fever.
- Splenomegaly.
- Jaundice.
Classification
Classified by etiology:[2]
- Primary, i.e. inherited:[3]
- Secondary:
Microscopic
Features:[1]
- Macrophages with phagocytosed:
- Erythrocytes.
- Leukocytes.
- Platelets.
Image(s): HLH (WC).
See also
References
- ↑ 1.0 1.1 Fisman DN (2000). "Hemophagocytic syndromes and infection". Emerging Infect. Dis. 6 (6): 601–8. PMC 2640913. PMID 11076718. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2640913/?tool=pubmed.
- ↑ Gupta S, Weitzman S (January 2010). "Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy". Expert Rev Clin Immunol 6 (1): 137–54. PMID 20383897.
- ↑ Nagai K, Yamamoto K, Fujiwara H, et al. (2010). "Subtypes of familial hemophagocytic lymphohistiocytosis in Japan based on genetic and functional analyses of cytotoxic T lymphocytes". PLoS ONE 5 (11): e14173. doi:10.1371/journal.pone.0014173. PMC 2994802. PMID 21152410. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2994802/.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 603552
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 603553