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Antiphospholipid syndrome
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ANTIPHOSPHOLIPID SYNDROME
AIMS
Antiphospholipid syndrome Pathophysiology diagnosis Treatment in pregnancy
APS/APLS/HUGHES SYNDROME
Autoimmune, hypercoagulable state caused by antibodies against cell-membrane phospholipids.
Leads to thrombosis and pregnancy related complications such as miscarriage, stillbirth, preterm delivery and severe preeclampsia
BURDEN OF DISEASE
CMACH:
ANTIPHOSPHOLIPID SYNDROME
Thrombosis (venous, arterial, microcirculation) and/or
Pregnancy morbidity >3 consecutive miscarriage <10 weeks >1 fetal death > 10 weeks >1 preterm birth <34 weeks with normal fetal
morphology due to pre-eclampsia or placental insufficiency
Combined with presence of aPL on 2 occasions, at least 6 weeks apart
DIAGNOSIS
Research criteria for defining the antiphospholipid syndrome. Adapted from Miyakis et al (2006). With permission, John Wiley & Sons,Inc. © 2006 International Society on Thrombosis and Haemostasis.
ANTIPHOSPHOLIPID ANTIBODIES (APL)
Heterogenous antibodies directed against anionic phospholipids/ phospholipid binding proteins (B2gp1, prothrombin, factor V,C,S, annexin-V)
Commonest are anticardiolipin antibody (aCL) and lupus anticoagulant (LA)
TYPES
Primary Alone Genetic marker: HLA-DR7
Secondary Associated with SLE or other autoimmune
disorders Genetic marker: HLA-DR2/3, HLA-B8 Race: Blacks, hispanic, asians, native americans
CLINICAL FEATURES
• Immune thrombocytopenia (ITP), haemolytic anaemia
• Cerebral: epilepsy, infarction, chorea, mirgraine, transverse myelitis
• Heart valve disease• Systemic/pulmonary hypertension• Livedo reticularis• Amorosis fugax• Leg ulcers
PATHOPHYSIOLOGY
DIAGNOSIS
PREGNANCY AND APLS
Risk of thrombosis exacerbated by the hypercoagulable pregnant state
Pre-existing ITP may worsen in pregnancy Increased risk of recc. Miscarriage, vascular
complications Complictions are less in
TREATMENT
RECOMENDATIONS IN PREGNANCY
TO TEST OR NOT TO TEST?
22 year old primip, presents with abdo pain and passing clots PV. Normally fit and well.
27 year old, para 1. 13/40, previous L arm DVT.
31 year old, 4 days postnatal. Previous TIA in postnatal period 1 yr previously.
18 year old with 2 previous miscarriages, presents with femerol DVT.