Antiphospholipid syndrome: Difference between revisions
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==See Also== | ==See Also== | ||
*[[DIC]], [[TTP]], [[HUS]], [[Microangiopathic Hemolytic Anemia (MAHA)]] | *[[DIC]], [[TTP]], [[HUS]], [[Microangiopathic Hemolytic Anemia (MAHA)]] | ||
*[[HELLP]], [[PNH]], [[HIT]] | *[[HELLP]], [[Paroxysmal Nocturnal Hemoglobinuria (PNH)]], [[HIT]] | ||
[[Category:Heme/Onc]] [[Category:Rheum]] | [[Category:Heme/Onc]] [[Category:Rheum]] |
Revision as of 23:06, 29 November 2013
Introduction
- APS definition (need 1 from each category):
- Presence of at least 1 of the following: DVT, arterial thrombosis, or pregnancy morbidity (eg fetal loss, preterm)
- Presence of at least 1 of the following antiphospholipid antibodies (aPL): lupus anticoagulant (LA), anticardiolipin (aCL), β2-glycoprotein-1 (β2-GP-1)
- APS can occur as a primary condition or in setting of underlying disease (eg SLE)
Pathophysiology
- Currently accepted theory: Susceptible pts (eg SLE) develop aPL after infection. After development of aPL, “second hit” stress required to develop full-blown APS. aPL affects coagulation by interacting with protein C, annexin V, platelets, proteases, tissue factor, and impairing finbrinolysis
- “Second hit” stressors: smoking, prolonged immobilization, pregnancy, exogenous estrogen, malignancy, nephrotic syndrome, HTN, hyperlipidemia
Diagnosis
- Presence of DVT, arterial thrombus, or pregnancy morbidity (eg fetal loss, preterm)
- Presence of aPL
Clinical Manifestations
- Thrombocytopenia, increased PT/INR and aPTT
- Microangiopathic Hemolytic Anemia (MAHA)
- DVT/PE
- Fetal loss
- Heart valve disease
- aPL-nephropathy
- Stroke/TIA, other neuro sx
- Livedo reticularis
Complications
- Catastrophic APS: widespread thrombotic disease w/ multiorgan failure precipitated by some stress (eg infection)
APS Treatment
- Anticoagulation (unfractionated heparin, LMWH, or warfarin)
- No benefit in treatment or prophy using ASA or plavix
- Add hydroxychloroquine if pt has SLE
- Warfarin contraindicated in pregnancy!
- IVIG, plasmapharesis, and steroids have not been proven to be of benefit in APS
Catastrophic APS Treatment
- Treat stress that preceipitated catastrophic APS (eg infection), anticoagulation, high dose steroids
- If evidence of microangiopathy (thrombocytopenia, MAHA), add IVIG and plasma exchange to above regimen