Difference between revisions of "Thrombotic microangiopathy"

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| Micro      = microvascular occlusion with "loose" intimal thickening - fluffy appearing intima, fibrin entrapped RBCs, +/-onion skin-like appearance (chronic change)  
| Micro      = microvascular occlusion with "loose" intimal thickening - fluffy appearing intima, fibrin entrapped RBCs, +/-onion skin-like appearance (chronic change)  
| Subtypes  = acute, chronic
| Subtypes  = acute, chronic
| LMDDx      = [[vasculitis]]
| LMDDx      = [[vasculitis]], small vessel
| Stains    =
| Stains    =
| IHC        =
| IHC        =
Line 27: Line 27:
| Prognosis  =
| Prognosis  =
| Other      =
| Other      =
| ClinDDx    =
| 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
| Tx        = dependent on underlying cause
}}
}}

Revision as of 20:15, 25 December 2013

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:

Chronic changes:

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.