Difference between revisions of "Hemophagocytic syndrome"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Hemophagocytic_syndrome_-_cropped_-_very_high_mag.jpg
| Width      =
| Caption    = Micrograph showing phagocytosed RBCs in hemophagocytic syndrome. [[H&E stain]].
| Synonyms  =
| Micro      = macrophages containing erythrocytes (red blood cells), leukocytes, platelets
| Subtypes  =
| LMDDx      = overlapping cells, [[emperipolesis]]
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      =
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = dependent on underlying cause
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Hemophagocytic syndrome''', also known as '''hemophagocytic lymphohistiocytosis''', is a rare condition often associated with viral infections.
'''Hemophagocytic syndrome''', also known as '''hemophagocytic lymphohistiocytosis''', is a rare condition often associated with viral infections.


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Features:<ref name=pmid11076718/>
Features:<ref name=pmid11076718/>
*Macrophages with phagocytosed:
*Macrophages with phagocytosed:
**Erythrocytes.
**[[Erythrocyte]]s.
**Leukocytes.
**Leukocytes.
**Platelets.
**Platelets.
DDx:
*Overlapping cells (red blood cells overlapping macrophages).
*[[Emperipolesis]].


===Images===
===Images===

Revision as of 05:52, 14 July 2015

Hemophagocytic syndrome
Diagnosis in short

Micrograph showing phagocytosed RBCs in hemophagocytic syndrome. H&E stain.

LM macrophages containing erythrocytes (red blood cells), leukocytes, platelets
LM DDx overlapping cells, emperipolesis
Prevalence rare
Prognosis dependent on underlying cause

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:

DDx:

  • Overlapping cells (red blood cells overlapping macrophages).
  • Emperipolesis.

Images

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.