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CCCEG Study Day

Hepatology and ICM Study in Cambridge
Tuesday 12th of December 2017

Seminars centered around care of the transplanted patient, managing acute liver failure, and predicting outcome in liver disease on the ICU.

East of England trainees free |£40 from elsewhere| £80 consultants

Email, or use contact form, to book your place.

RCoA CPD points applied for.


Thrombotic Thrombocytopenic Purpura

By Dr Rob Hackett

TTP consists of the pentad of:

  • Microangiopathic haemolytic anaemia
  • Thrombocytopenic purpura
  • Neurologic abnormalities
  • Fever
  • Renal disease

Untreated mortality = 90%

Treatment with plasma exchange = 10-20%

Lack of plasma protease – A Disintegrinlike And Metalloprotease with ThromboSpondin type 1 motif 13 (ADAMTS13) – responsible for the breakdown of ultralarge vWF multimers.

Platelet-vWF complexes form small blood clots which circulate in the blood vessels and cause shearing of red blood cells, resulting in their rupture – Haemolytic anaemia and Schistocyte formation.

Similar to HUS but differentiated by coagulation screen results.

In TTP, the organ most obviously affected is the brain; in HUS, the kidney.

Relapses are not uncommon, occurring in 13-36% of patients.

Severe bleeding from thrombocytopenia is unusual, although petechiae are common

Laboratory Investigations:

  • Measuring ADAMTS13 activity level may aid in diagnosis.
  • FBC – ? Hb + Platelets
  • Bilirubin + LDH
  • Blood film
  • Coagulation screen
  • U+E

Tx of choice for TTP is plasma exchange with fresh frozen plasma

Transfusion is contraindicated in thrombotic TTP, as it fuels the coagulopathy.

Corticosteroids may be used in refractory patients

Rituximab, a monoclonal antibody aimed at the CD20 molecule on B lymphocytes – destruction of B cells reduces the production of the protease inhibitor.