10. Antiphospholipid syndrome

Question 1

what is APS?

Answer 1

autoantibody- mediated acquired thrombophilia

characterized by recurrent arterial or venous thrombosis and pregnancy morbidity

Question 2

what triggers antibodies to PL-binding proteins in APS?

Answer 2

unknown
disease exacerbation is induced by infections, major physical stress (surgery) and discontinuation of anticoagulant treatment

Question 3

what is Catastrophic APS?

Answer 3

acute thrombotic microangiopathy with multiorgan damage (≥ 3 organs involved)

Question 3

what is Catastrophic APS?

Answer 3

acute thrombotic microangiopathy with multiorgan damage (≥ 3 organs involved)

Question 4

treatment of Catastrophic APS

Answer 4

plasma exchange
cyclophosphamide
rituximab

Question 5

clinical manifestations of APS

Answer 5

1. venous thrombosis
2. arterial thrombosis
3. obstetric manifestations
4. thrombocytopenia, hemolytic anemia, livedo reticularis

Question 6

definitive diagnosis of antiphospholipid syndrome requires?

Answer 6

≥ 1 clinical criteria and ≥ 1 laboratory criteria

Question 7

what is lupus anticoagulant?

Answer 7

Ab against phospholipid and proteins associated with cell membrane

↑ PTT that does not correct with mixing study
but does correct with excess phospholipids or platelets

misnomer as it actually is prothrombotic antibody

Question 8

are DOAC’s effective in APS?

Answer 8

Less effective than warfarin

especially those with history of arterial events

Question 9

after the first thrombotic event, APS patients should be placed on?

Answer 9

warfarin for life (INR 2-3)

in combination with low-dose aspirin daily