Sunday, November 29, 2009

TTP









We discussed Thrombotic Thrombocytopenic Purpura (TTP), a very important condition to recognize and institute treatement because of its 90% untreated mortality.

Classic pentad:
1) Thrombocytopenia
2) Microangiopathic hemolytic anemia (MAHA; recognized by hemolysis with RBC fragments-shown above)
3) Neurological signs/symptoms
4) Renal failure
5) Fever

HOWEVER,
the full pentad is seen late in the course. Thrombocytopenia and MAHA without another cause is generally enough to start therapy

Pathologically, thrombin/platelet depositions in organs.

Pathogenesis: ADAMTS13 is a metalloproteinase which degrades von Willebrand's factor. Loss of this enzyme (acquired or genetic) causes large multimers of vWF to accumulate, activating platelets and causing microvascular clotting.

Causes of acquired TTP:
Infection: E.Coli 0157:H7, which causes colonic vascular injury and may induce TTP.
Drugs: ticlopidine, clopidogrel, cyclosporin
Cancer: TTP is assoc with Br, GI, pancreatic, prostate
Autoimmune disease: SLE (most common), RA, dermatomyositis, others.


Treatment:
Plasma exchange: Deficiency in ADAMTS13 is corrected, and antibody against it is removed. Done daily until LDH and PLT normalize. Large volumes of FFP may be transfused until transfer to a PLEX centre

Steroids- prednisone 1mg/kg/d PO or solumedrol 125mg IV BID as adjunct to PLEX.

PLT transfusion- not recommended unless life-threatening bleeding

Links:

Click here for the NEJM paper from Ottawa first showing the substantial benefit of PLEX

Click here for a NEJM review article on TTP

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