Thrombotic thrombocytopaenic purpura (TTP)
Last updated: Thursday, 20, January, 2011
TTP is a rare disorder, caused by a congenital or aquired 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 aquired 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. Its treatment is a medical emergency.
FBC, blood film (red cell fragmentation), platelet count; creatinine, urea, electrolytes. Urine - urinalysis, microscopy. ADAMTS 13 assays are performed by some laboratories. Patients with phospholipid antibody syndrome may present with features resembling TTP.
See also Haemolytic uraemic syndrome
|Increased reticulocytes, bilirubin and LDH are expected with anaemia.|
|Variable but often severe.|
|Variable at presentation and may be absent. See also Pyrexia of unknown origin|
Fluctuating neurological signs
Renal impairment, including