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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 = primary, secondary | |||
| LMDDx = overlapping cells, [[emperipolesis]] | |||
| Stains = | |||
| IHC = | |||
| EM = | |||
| Molecular = FHL1 mutation or FHL2 mutation in primary form | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[lymph nodes]], [[liver]], [[spleen]], [[bone marrow]], others | |||
| Assdx = only in ''secondary'' form (EBV infection, malignancy, rheumatologic disease) | |||
| Syndromes = | |||
| Clinicalhx = +/-consanguinity for ''primary'' form (autosomal recessive) | |||
| Signs = fever, [[splenomegaly]], jaundice | |||
| Symptoms = | |||
| Prevalence = rare | |||
| Bloodwork = +/-EBV serology (secondary form), +/-HIV serology (secondary form) | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = dependent on underlying cause | |||
| Other = | |||
| ClinDDx = malignancy | |||
| Tx = dependent on underlying cause | |||
}} | |||
'''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. | ||
| Line 11: | Line 42: | ||
*[[Liver]]. | *[[Liver]]. | ||
*[[Spleen]]. | *[[Spleen]]. | ||
*Bone marrow. | *[[Bone marrow]]. | ||
==Classification== | ==Classification== | ||
| Line 29: | Line 60: | ||
Features:<ref name=pmid11076718/> | Features:<ref name=pmid11076718/> | ||
*Macrophages with phagocytosed: | *Macrophages with phagocytosed: | ||
** | **[[Erythrocyte]]s. | ||
**Leukocytes. | **Leukocytes. | ||
**Platelets. | **Platelets. | ||
DDx: | |||
*[ | *Overlapping cells (red blood cells overlapping macrophages). | ||
*[[Emperipolesis]] in the context of other pathology. | |||
===Images=== | |||
<gallery> | |||
Image:Hemophagocytic_syndrome_-_cropped_-_very_high_mag.jpg | Hemophagocytic syndrome - cropped - very high mag. (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)]. | ||
==See also== | ==See also== | ||
*[[Hematopathology]]. | *[[Hematopathology]]. | ||
*[[Myospherulosis]]. | |||
*[[Emperipolesis]]. | |||
==References== | ==References== | ||