Thrombotic microangiopathy

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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.