Difference between revisions of "Thrombotic microangiopathy"

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'''Thrombotic microangiopathy''' is a group of disorders affecting small blood vessels. ''Microangiopathic'' is a disease affecting small blood vessels.<ref>URL: [http://www.mercksource.com/pp/us/cns/cns_hl_dorlands_split.jsp?pg=/ppdocs/us/common/dorlands/dorland/one/000005089.htm http://www.mercksource.com/pp/us/cns/cns_hl_dorlands_split.jsp?pg=/ppdocs/us/common/dorlands/dorland/one/000005089.htm]. Accessed on: 9 September 2010.</ref>
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Acute thrombotic microangiopathy - very high mag.jpg
| Width      =
| Caption    = Acute thrombotic microangiopathy. [[H&E stain]].
| Synonyms  =
| Micro      = microvascular occlusion with "loose" intimal thickening - fluffy appearing intima, fibrin entrapped RBCs, +/-onion skin-like appearance (chronic change)
| Subtypes  = acute, chronic
| LMDDx      = [[vasculitis]], small vessel
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[blood vessels]] - esp. [[kidney]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    = DDx for TMA: [[malignant hypertension]], [[DIC]], [[APLA]] in [[SLE]], [[scleroderma]] renal crisis, [[HIV]] associated [[TTP]], reaction to chemotherapy, [[calcineurin inhibitor toxicity]]
| Tx        = dependent on underlying cause
}}
'''Thrombotic microangiopathy''', abbreviated '''TMA''', is a group of disorders affecting small blood vessels. ''Microangiopathic'' is a disease affecting small [[blood vessels]].<ref>URL: [http://www.mercksource.com/pp/us/cns/cns_hl_dorlands_split.jsp?pg=/ppdocs/us/common/dorlands/dorland/one/000005089.htm http://www.mercksource.com/pp/us/cns/cns_hl_dorlands_split.jsp?pg=/ppdocs/us/common/dorlands/dorland/one/000005089.htm]. Accessed on: 9 September 2010.</ref>


==Differential diagnosis==
===Clinical===
The grouping includes:<ref>URL: [http://emedicine.medscape.com/article/779218-overview http://emedicine.medscape.com/article/779218-overview]. Accessed on: 8 November 2010.</ref><ref name=pmid15915311>{{cite journal |author=Mayer SA, Aledort LM |title=Thrombotic microangiopathy: differential diagnosis, pathophysiology and therapeutic strategies |journal=Mt. Sinai J. Med. |volume=72 |issue=3 |pages=166–75 |year=2005 |month=May |pmid=15915311 |doi= |url=}}</ref>
The grouping includes:<ref>URL: [http://emedicine.medscape.com/article/779218-overview http://emedicine.medscape.com/article/779218-overview]. Accessed on: 8 November 2010.</ref><ref name=pmid15915311>{{cite journal |author=Mayer SA, Aledort LM |title=Thrombotic microangiopathy: differential diagnosis, pathophysiology and therapeutic strategies |journal=Mt. Sinai J. Med. |volume=72 |issue=3 |pages=166–75 |year=2005 |month=May |pmid=15915311 |doi= |url=}}</ref>
*[[Hemolytic uremic syndrome]].
*[[Hemolytic uremic syndrome]].
*[[Thrombotic thrombocytopenic purpura]] (TTP).  
*[[Thrombotic thrombocytopenic purpura]] (TTP).  
Others:<ref name=pmid20186056>{{Cite journal  | last1 = Benz | first1 = K. | last2 = Amann | first2 = K. | title = Thrombotic microangiopathy: new insights. | journal = Curr Opin Nephrol Hypertens | volume = 19 | issue = 3 | pages = 242-7 | month = May | year = 2010 | doi = 10.1097/MNH.0b013e3283378f25 | PMID = 20186056 }}</ref>
*[[Malignant hypertension]].<ref name=pmid18497467>{{Cite journal  | last1 = Zhang | first1 = B. | last2 = Xing | first2 = C. | last3 = Yu | first3 = X. | last4 = Sun | first4 = B. | last5 = Zhao | first5 = X. | last6 = Qian | first6 = J. | title = Renal thrombotic microangiopathies induced by severe hypertension. | journal = Hypertens Res | volume = 31 | issue = 3 | pages = 479-83 | month = Mar | year = 2008 | doi = 10.1291/hypres.31.479 | PMID = 18497467 }}</ref>
*[[DIC]].
*[[APLA]]<ref name=pmid9814675>{{Cite journal  | last1 = Asherson | first1 = RA. | title = The catastrophic antiphospholipid syndrome, 1998. A review of the clinical features, possible pathogenesis and treatment. | journal = Lupus | volume = 7 Suppl 2 | issue =  | pages = S55-62 | month =  | year = 1998 | doi =  | PMID = 9814675 }}</ref> in [[SLE]].
*[[Scleroderma]] renal crisis.
*[[HIV]] associated [[TTP]].
*Chemotherapy.
*[[Calcineurin inhibitor toxicity]].
Notes:
*The above cannot be differentiated on a histomorphologic basis.
===Histologic===
The histomorphologic [[differential diagnosis]] is:
*[[Vasculitis]].
**Inflammatory cells within the vessel wall.
**Vessel wall injury, i.e. [[necrosis]].
==General==
*Underlying pathology: endothelial cell damage.


==Microscopic==
==Microscopic==
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#Microvascular occlusion.<ref name=Ref_PBoD8_670>{{Ref PBoD8|670}}</ref>
#Microvascular occlusion.<ref name=Ref_PBoD8_670>{{Ref PBoD8|670}}</ref>
#"Loose" intimal thickening; fluffy appearing intima.
#"Loose" intimal thickening; fluffy appearing intima.
#*May be have an onion skin-like appearance.
#Fibrin entrapped RBCs.
#Fibrin entrapped RBCs.


Notes:  
Notes:  
*Microvascular occlusion is non-specific -- also seen in DIC.
*The last two (#2 and #3) are useful for discrimination from endarteritis.<ref name=Ref_FoRP>{{Ref FoRP|204}}</ref>
*The last two (#2 and #3) are useful for discrimination from endarteritis.<ref name=Ref_FoRP>{{Ref FoRP|204}}</ref>
*Early finding: endothelial cell swelling.
===Images===
www:
*[http://www.nature.com/ki/journal/v60/n3/fig_tab/4492492f5.html Thrombotic microangiopathy (nature.com)].<ref name=pmid11532079>{{Cite journal  | last1 = Ruggenenti | first1 = P. | last2 = Noris | first2 = M. | last3 = Remuzzi | first3 = G. | title = Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. | journal = Kidney Int | volume = 60 | issue = 3 | pages = 831-46 | month = Sep | year = 2001 | doi = 10.1046/j.1523-1755.2001.060003831.x | PMID = 11532079 }}</ref>
*[http://path.upmc.edu/cases/case683.html Probable APLA syndrome - several images (upmc.edu)].
Acute changes:
<gallery>
Image: Acute thrombotic microangiopathy - high mag.jpg | High mag.
Image: Acute thrombotic microangiopathy - very high mag.jpg | Very high mag.
Image: Acute thrombotic microangiopathy - pas - high mag.jpg | High mag.
Image: Acute thrombotic microangiopathy - pas - very high mag.jpg | Very high mag.
</gallery>
Chronic changes:
<gallery>
Image: Thrombotic microangiopathy - intermed mag.jpg | Intermed. mag.
Image: Thrombotic microangiopathy - high mag.jpg | High mag.
Image: Thrombotic microangiopathy - very high mag.jpg | Very high mag.
</gallery>


==See also==
==See also==
*[[Vasculitides]].
*[[Vasculitides]].
*[[Medical renal diseases]].
*[[Medical renal diseases]].
*[[Hyperplastic arteriolosclerosis]].


==References==
==References==
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[[Category:Cardiovascular pathology]]
[[Category:Cardiovascular pathology]]
[[Category:Diagnosis]]
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