Difference between revisions of "Thrombotic microangiopathy"
Jump to navigation
Jump to search
(→Differential diagnosis: more) |
m (w) |
||
| (21 intermediate revisions by the same user not shown) | |||
| Line 1: | Line 1: | ||
'''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 7: | Line 39: | ||
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> | 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]]. | *[[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]]. | ||
*Chemotherapy. | *Chemotherapy. | ||
*Calcineurin inhibitor toxicity. | *[[Calcineurin inhibitor toxicity]]. | ||
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== | ||
| Line 32: | Line 70: | ||
*Early finding: endothelial cell swelling. | *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://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== | ||
| Line 44: | Line 99: | ||
[[Category:Cardiovascular pathology]] | [[Category:Cardiovascular pathology]] | ||
[[Category:Diagnosis]] | |||
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
Others:[4]
- Malignant hypertension.[5]
- DIC.
- APLA[6] 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
Features:[7]
- Microvascular occlusion.[8]
- "Loose" intimal thickening; fluffy appearing intima.
- May be have an onion skin-like appearance.
- 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
- ↑ 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.
- ↑ URL: http://emedicine.medscape.com/article/779218-overview. Accessed on: 8 November 2010.
- ↑ Mayer SA, Aledort LM (May 2005). "Thrombotic microangiopathy: differential diagnosis, pathophysiology and therapeutic strategies". Mt. Sinai J. Med. 72 (3): 166–75. PMID 15915311.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 7.0 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.
- ↑ 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.
- ↑ 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.