What are the differential diagnoses for TTP?

Disease Other Thrombotic Microangiopathies DIC HELLP Other ITP HIT Heart Valve Haemolysis Evan's Syndrome Endocarditis Catastrophic anti-phospholipid syndrome Pathophysiology Inflammatory mediated consumptive coagulopathy Inflammatory mediated consumptive coagulopathy...

How do you differentiate TTP and haemolytic uraemic syndrome (HUS)?

Can be difficult as share similar clinical and pathophysiological features Differentiating clinical features include: ↑ presence of focal neurological symptoms in TTP ↑ presence of renal failure in HUS Typical HUS: Associated with exposure to shiga toxin – may...

How is TTP Diagnosed?

The hallmark of TTP is absence of ADAMTS13 activity A level <5–10% is now required to confirm the...

How can ADAMTS13 activity deficiency be detected?

ADAMTS13 activity is tested using specialist functional assays which are conducted at reference centres: Principle involves degradation of vWF substrate (either full length or small peptides) The value is expressed as a % compared against activity from normal pooled...

What is the management of TTP?

Key PrinciplesEarly resuscitation and supportive careUrgent and rapid treatment or transfer to specialist centre (Treat as urgent as aneurysm) Avoidance of platelet transfusionEarly plasma exchange with FFPImmunosuppressive therapyTherapy to reduce thrombosis Initial...