TTP

Question 1

1) Clinical features of immune TTP 2) common clinical scenario

Answer 1

Initial symptoms (fatigue, dyspnea, petechia, abdominal pain/ nausea/vomiting (45%), headache/confusion (20%) - typically mild symptoms at onset) + abnormal or normal (more commonly) kidney function (AKI uncommon), often comorbid autoimmune disorders + typically previously healthy person + median age 40 + females more commonly (75%) + increased frequency in pregnancy

Question 2

Primary cause of death in TTP

Answer 2

myocardial infarction

Question 3

Diagnostic cutoff for ADAMTS13 level in TTP

Answer 3

<10%

Question 4

caplacizumab mechanism

Answer 4

blocks platelet interaction with von willebrand factor

Question 5

congenital TTP treatment

Answer 5

• plasma infusion
• recombinant ADAMTS13

Question 6

Screening required for patients with history of TTP

Answer 6

• lifelong cardiovascular screening and risk stratification (major cause of mortality)