Difference between revisions of "Hemophagocytic syndrome"

From Libre Pathology
Jump to navigation Jump to search
(→‎Microscopic: +images)
Line 22: Line 22:
#**FHL2.<ref>{{OMIM|603553}}</ref>
#**FHL2.<ref>{{OMIM|603553}}</ref>
#Secondary:
#Secondary:
#*Infection, e.g. [[EBV]],<ref name=pmid20819601>{{cite journal |author=Jin YK, Xie ZD, Yang S, Lu G, Shen KL |title=Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: a retrospective study of 78 pediatric cases in mainland of China |journal=Chin. Med. J. |volume=123 |issue=11 |pages=1426–30 |year=2010 |month=June |pmid=20819601 |doi= |url=}}</ref> [[HIV]].  
#*Infection, e.g. [[EBV]] (most common<ref>{{Ref WMSP|576}}</ref>),<ref name=pmid20819601>{{cite journal |author=Jin YK, Xie ZD, Yang S, Lu G, Shen KL |title=Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: a retrospective study of 78 pediatric cases in mainland of China |journal=Chin. Med. J. |volume=123 |issue=11 |pages=1426–30 |year=2010 |month=June |pmid=20819601 |doi= |url=}}</ref> [[HIV]].  
#*Malignancy.  
#*Malignancy.  
#*Rheumatologic condition.
#*Rheumatologic condition.

Revision as of 00:20, 10 February 2012

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis, is a rare condition often associated with viral infections.

Clinical

Features:[1]

Involved organs:

Classification

Classified by etiology:[2]

  1. Primary, i.e. inherited:[3]
    • Etiology: dysfunction of cytotoxic T lymphocytes with macrophage activating cytokines.
    • Autosomal recessive.
    • Two genes identified:
  2. Secondary:
    • Infection, e.g. EBV (most common[6]),[7] HIV.
    • Malignancy.
    • Rheumatologic condition.

Microscopic

Features:[1]

  • Macrophages with phagocytosed:
    • Erythrocytes.
    • Leukocytes.
    • Platelets.

Images:

See also

References

  1. 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.
  2. 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.
  3. 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/.
  4. Online 'Mendelian Inheritance in Man' (OMIM) 603552
  5. Online 'Mendelian Inheritance in Man' (OMIM) 603553
  6. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 576. ISBN 978-0781765275.
  7. Jin YK, Xie ZD, Yang S, Lu G, Shen KL (June 2010). "Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: a retrospective study of 78 pediatric cases in mainland of China". Chin. Med. J. 123 (11): 1426–30. PMID 20819601.