Thrombotic Thrombocytopenic Purpura

• occurs in adults

Syndrome of

• renal impairment

• fragmented red cells

• decreased platelets

• fever

• neurological features

  • headache

  • loss of consciousness

Associations

• autoimmune conditions

  • SLE

• pregnancy

• infection

Pathology

• deficiency of protease that cleaves von Willebrand Factor

  • congenital

  • antibody

Treatment

• plasma exchange

• fresh frozen plasma (possibly depleted of cryoprecipitate) as replacement

  • solvent treated less likely to transmit infection

• antiplatelet drugs

• corticosteroids

• splenectomy

• vincristine

Monitoring Response

• haemoglobin

• reticulocytes

• lactate dehydrogenase

• platelet count

• plasma bilirubin

• vWF multimers

Prognosis

• may be fatal

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