Difference between revisions of "Hemophagocytic syndrome"

From Libre Pathology
Jump to navigation Jump to search
(→‎Classification: more in genetic type)
Line 6: Line 6:
*[[Splenomegaly]].
*[[Splenomegaly]].
*Jaundice.
*Jaundice.
Involved organs:
*[[Lymph nodes]].
*[[Liver]].
*[[Spleen]].
*Bone marrow.


==Classification==
==Classification==

Revision as of 17:21, 9 February 2011

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, HIV.
    • Malignancy.
    • Rheumatologic condition.

Microscopic

Features:[1]

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

Image(s): HLH (WC).

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