Condition- Anti-phospholipid syndrome Flashcards
Define anti-phospholipid syndrome
Immune mediated thrombosis characterised by the presence of antiphospholipid antibodies leading to arterial and venous thrombosis and recurrent foetal miscarriage and thrombocytopenia
Which three antibodies are present in patients with antiphospholipid syndrome?
- Anti-B-Glycoprotein-1 antibodies
- Anti-cardiolipin antibodies
- Lupus Anticoagulant
Anti-phospholipid syndrome can be a primary or secondary disease. Which condition does it often develop secondary to?
SLE
List some infectious diseases that provide the ‘second hit’ for the development of anti-phospholipid syndrome in pre-disposed individuals
- Syphylis
- Malaria
- Hep C
- HIV
What is the function of the B-GP-1 protein?
- B-Glycoprotein-1 is a cell surface protein bound to phospholipids
- It prevents platelet agglution via Glp2b/3a
Describe how antiphospholipid syndrome causes hypercoagulability and predisposes patients to thromboses
- Anti-B-GP-1 antibodies in antiphospholipid syndrome target B-GP-1 protein
- => reduced inhibition of platelet agglutination = Increased agglutination and clotting
State the main way a female with antiphospholipid syndrome presents
During pregnancy with miscarriage- may have recurrent miscarriages
List some of the presenting symptoms of Anti-phospholipid syndrome
- Arterial Thrombosis: Stroke, MI
- Venous Thrombosis: DVT, PE
- Miscarriage
- Kidney: AKI
State a dermatological sign of Anti-phospholipid syndrome
Livedo Reticularis- caused by swelling of venules due to obstruction of capillaries by small blood clots
Diagnosis of aPL syndrome can be confirmed by…
The presence of:
- Anti-B-GP-1 antibodies
- Lupus anticoagulant
- Anticardiolipin antibodies
What might you see in the bloods of someone with aPL syndrome?
- Low platelets (due to autoimmune destruction)
- U+Es: high Cr, high urea due to aPL nephropathy
- Prolonged APTT - a paradoxical rise due to lupus anti-coagulant
- serum antibodies
