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
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:
1 comment :
I started on COPD Herbal treatment from Ultimate Health Home, the treatment worked incredibly for my lungs condition. I used the herbal treatment for almost 4 months, it reversed my COPD. My severe shortness of breath, dry cough, chest tightness gradually disappeared. Reach Ultimate Health Home via their website at www.ultimatelifeclinic.com I can breath much better and It feels comfortable!
Post a Comment