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==
==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]].
Line 9: Line 41:
*[[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>
*[[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]].
*[[DIC]].
*[[APLA]]<ref>{{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]].
*[[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.
*[[Scleroderma]] renal crisis.
*[[HIV]] associated [[TTP]].
*[[HIV]] associated [[TTP]].
Line 17: Line 49:
Notes:  
Notes:  
*The above cannot be differentiated on a histomorphologic basis.
*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==
==General==
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*Early finding: endothelial cell swelling.
*Early finding: endothelial cell swelling.


Images:
===Images===
*www:
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://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>
*[[WC]]:
*[http://path.upmc.edu/cases/case683.html Probable APLA syndrome - several images (upmc.edu)].
**[http://commons.wikimedia.org/wiki/File:Thrombotic_microangiopathy_-_intermed_mag.jpg TMA - intermed. mag. (WC)].
 
**[http://commons.wikimedia.org/wiki/File:Thrombotic_microangiopathy_-_very_high_mag.jpg TMA - very high mag. (WC)].
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==

Latest revision as of 15:35, 11 January 2014

Thrombotic microangiopathy
Diagnosis in short

Acute thrombotic microangiopathy. H&E stain.
LM microvascular occlusion with "loose" intimal thickening - fluffy appearing intima, fibrin entrapped RBCs, +/-onion skin-like appearance (chronic change)
Subtypes acute, chronic
LM DDx vasculitis, small vessel
Site blood vessels - esp. kidney
Clin. DDx DDx for TMA: malignant hypertension, DIC, APLA in SLE, scleroderma renal crisis, HIV associated TTP, reaction to chemotherapy, calcineurin inhibitor toxicity
Treatment 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.[1]

Differential diagnosis

Clinical

The grouping includes:[2][3]

Others:[4]

Notes:

  • The above cannot be differentiated on a histomorphologic basis.

Histologic

The histomorphologic differential diagnosis is:

General

  • Underlying pathology: endothelial cell damage.

Microscopic

Features:[7]

  1. Microvascular occlusion.[8]
  2. "Loose" intimal thickening; fluffy appearing intima.
    • May be have an onion skin-like appearance.
  3. Fibrin entrapped RBCs.

Notes:

  • The last two (#2 and #3) are useful for discrimination from endarteritis.[7]
  • Early finding: endothelial cell swelling.

Images

www:

Acute changes:

  • High mag.

  • Very high mag.

  • High mag.

  • Very high mag.

Chronic changes:

  • Intermed. mag.

  • High mag.

  • Very high mag.

See also

References

  1. ↑ URL: 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.
  2. ↑ URL: http://emedicine.medscape.com/article/779218-overview. Accessed on: 8 November 2010.
  3. ↑ Mayer SA, Aledort LM (May 2005). "Thrombotic microangiopathy: differential diagnosis, pathophysiology and therapeutic strategies". Mt. Sinai J. Med. 72 (3): 166–75. PMID 15915311.
  4. ↑ Benz, K.; Amann, K. (May 2010). "Thrombotic microangiopathy: new insights.". Curr Opin Nephrol Hypertens 19 (3): 242-7. doi:10.1097/MNH.0b013e3283378f25. PMID 20186056.
  5. ↑ Zhang, B.; Xing, C.; Yu, X.; Sun, B.; Zhao, X.; Qian, J. (Mar 2008). "Renal thrombotic microangiopathies induced by severe hypertension.". Hypertens Res 31 (3): 479-83. doi:10.1291/hypres.31.479. PMID 18497467.
  6. ↑ Asherson, RA. (1998). "The catastrophic antiphospholipid syndrome, 1998. A review of the clinical features, possible pathogenesis and treatment.". Lupus 7 Suppl 2: S55-62. PMID 9814675.
  7. ↑ 7.0 7.1 Fogo, Agnes; Bruijn, Jan A.; Cohen, Arthur H.; Colvin, Robert B.;Jennette, J. Charles (2006). Fundamentals of Renal Pathology (1st ed.). Springer. pp. 204. ISBN 978-0387311265.
  8. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 670. ISBN 978-1416031215.
  9. ↑ Ruggenenti, P.; Noris, M.; Remuzzi, G. (Sep 2001). "Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura.". Kidney Int 60 (3): 831-46. doi:10.1046/j.1523-1755.2001.060003831.x. PMID 11532079.
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