Thrombotic thrombocytopenic purpura

Keywords: TTP

Presentation

Appropriate Tests

 

TTP is a rare disorder, caused by a congenital or acquired deficiency of the Von Willebrand factor (VWF) cleaning protease ADAMTS-13. There is a response to plasma exchange (in congenital disorders) and to plasmapheresis (in acquired disorders). There is a high mortality rate untreated. Its hallmarks are thrombocytopenia (often severe), red cell fragmentation and evidence of haemolysis, with variable CNS and renal dysfunction. Treatment is a medical emergency.

Full blood count, Blood film (red cell fragmentation); Creatinine, Urea, Electrolytes. Urine - Urinalysis, microscopy. ADAMTS-13 activity assay is performed by some laboratories. 

See also Haemolytic uraemic syndrome

Microangiopathic haemolysis

Increased Reticulocyte count, Bilirubin and Lactate dehydrogenase are expected with anaemia.

Thrombocytopenia

Variable but often severe.

Fever

Variable at presentation and may be absent. See also Pyrexia of unknown origin

Fluctuating neurological signs

Variable.

Renal impairment, including

Variable.

  • Proteinuria

 

  • Haematuria

 

  • Renal failure - acute