TTP Flashcards
Prognosis of TTP
Haemotological emergnecy
10-20% mortality, higher if not treated - 80-90%
What is TTP
Thrombotic microangiography
Haemolytic anaemia + ass low platelets
End organ damage from microscopic blood clots capillaries and small arteries
What causes TTP
ADAMTS13 protein deficiency
Types of TTP
Congential - young children or pregnancy
Aquired - immune - autoantibodies
What does ADAMTS13do
Cleaves VWF
What does AMATS13 deficiency cause
No cleaving of VWF -> long strings/multimers of VWF
Platelets adhere to VWF multimers -> clots -> widespread microthrombi
What need to diagnose TTP
Microangiographic haemolytic anaemia
Low plateelts <50
Microvascular thrombosis
ADAMTS13 <10% =/- antiADAMTS13 igG
Associations/underlying causes
How diagnosie Microangiopathic haemolytic anaemia
Ecidence of haemolyiss
Fragments of blood film
Schistocytes
Microvascular thrombus where present and how
Can be anywhere but
Renal impairment - mild
Neurological - headaches, intermittent confusion, reduced GCS, coma, seizures
Cardiac -chest pain, HF
Diagnostic test for TTP
ADAMTS13 <10%
antiADAMTS13 igG autoantibodies
Underlying causes TTP
AI conditions
Viral infections
eg HIV, pancreatitis, medications, pregnancy, malignancy
Treat TTP
TRANSFER TO CENTRE ideal within 4 hours, max 8 hours
can do 24/7 apheresis
Plasma exchange
High dose steroids
Rituximab
Caplacizumab - targets VWF inhibits platelet interactions
Ongoing careTTP
Relapsing remtitting course - require further treatment to prevent relapse
Look for end organ damage signs - MRI head, ECHO
Monitor ADAMTS13 activity - every 1-2 weeks in first month, increase to once a month, every other month
Congenital TTP
What does Congenital TTP need treat
Regular ocotplas infusons to replace ADAMTS13
When suspect TTP over other TMAs
Higher degree TP
Absence ass coagulopathy
More prominent neuro symptoms
