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 Guide Editors
 Editor In Chief
    Joel E. Gallant, MD, MPH

Pharmacology Editor
    Paul Pham, PharmD, BCPS

Zambia Guideline Team
   Peter Mwaba MMed PhD FRCP
   Alywn Mwinga MMed
   Isaac Zulu MMed MPH
   Velepie Mtonga MMed
   Albert Mwango MBChB
   Jabbin Mulwanda MMed FCS
 

 

 

Diagnosis>Organ System>
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Thrombotic thrombocytopenic purpura

Richard D. Moore, M.D.
08-05-2008

  • No data on epidemiology of TTP in Zambia or the rest of sub-Saharan Africa.
  • Plasma exchange not available in Zambia.
Zambia Information Author: Larry William Chang, MD, MPH

CLINICAL

  • Multisystem disease caused by platelet thrombi in various organs.
  • Thrombocytopenia, hemolytic anemia, renal impairment, fever (can be high), and neurologic abnormalities (agitation, disorientation, headache early, late focal deficits, seizure, coma).
  • Relatively rare; mortality <10% if recognized and treated early.
  • Associated with early and late HIV, malignancy, chemotherapy, pregnancy.
  • Drugs associated with TTP: quinine, ticlopidine, cyclosporine.

DIAGNOSIS

  • Low platelet count (5000-100,000), hemolytic anemia (causing high indirect bilirubin and high LDH), fragmented RBCs on peripheral smear (schistocytes, spherocytes), azotemia, neurologic dysfunction.
  • Low von Willebrand factor-cleaving protease level (<5-10%) and presence of inhibitor may be demonstrated.

TREATMENT

Treatment of TTP

  • Early Dx and urgent treatment required. Mortality rate 60-80% without treatment.
  • Plasma exchange with fresh frozen plasma (FFP) daily until normal PLT count and LDH. Average of 7-16 exchanges may be needed. HIV+ may respond better than HIV- to FFP.
  • HAART use in addition to plasma exchange is beneficial
  • Mild disease may respond to prednisone at 200 mg/d, but plasma exchange needed if no response within 48 hrs. Prednisone can be added if poor response to initial treatment with plasma exchange.
  • Aspirin and dipyridamole not effective if given alone.
  • FFP daily until normal platelets and LDH.

Drug Comments

DrugRecommendations/Comments
.

FOLLOW UP

Relapse

  • Relapse can occur, usually within 60 days (~10%), but can occur years later.
  • Plasma exchange usually effective for relapse.

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