Difference between revisions of "Hemophagocytic syndrome"

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**Platelets.
**Platelets.


Images:
===Images===
*[http://en.wikipedia.org/wiki/File:Hemophagocytic_syndrome_-_cropped_-_very_high_mag.jpg Hemophagocytic syndrome - cropped - very high mag. (WC)].
<gallery>
*[http://en.wikipedia.org/wiki/File:Hemophagocytic_syndrome_-_very_high_mag.jpg Hemophagocytic syndrome - very high mag. (WC)].
Image:Hemophagocytic_syndrome_-_cropped_-_very_high_mag.jpg | Hemophagocytic syndrome - cropped - very high mag. (WC)
*[http://en.wikipedia.org/wiki/File:Haemophagocytic_lymphohistiocytosis_Bone_marrow.JPG HLH (WC)].
Image:Hemophagocytic_syndrome_-_very_high_mag.jpg | Hemophagocytic syndrome - very high mag. (WC)
Image:Haemophagocytic_lymphohistiocytosis_Bone_marrow.JPG | HLH (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case601.html Hemophagocytic syndrome - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case601.html Hemophagocytic syndrome - several images (upmc.edu)].


Revision as of 23:52, 19 December 2013

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 infectious etiology[6]),[7] HIV.
    • Malignancy.
    • Rheumatologic condition.

Microscopic

Features:[1]

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

Images

  • Hemophagocytic syndrome - cropped - very high mag. (WC)

  • Hemophagocytic syndrome - very high mag. (WC)

  • HLH (WC)

www:

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.
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