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ANTIPHOSPHOLIPID SYNDROME

Antiphospholipid syndrome

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Antiphospholipid syndrome

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Page 1: Antiphospholipid syndrome

ANTIPHOSPHOLIPID SYNDROME

Page 2: Antiphospholipid syndrome

AIMS

Antiphospholipid syndrome Pathophysiology diagnosis Treatment in pregnancy

Page 3: Antiphospholipid syndrome

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

Page 4: Antiphospholipid syndrome

BURDEN OF DISEASE

CMACH:

Page 5: Antiphospholipid syndrome

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

Page 6: Antiphospholipid syndrome

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.

Page 7: Antiphospholipid syndrome

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)

Page 8: Antiphospholipid syndrome

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

Page 9: Antiphospholipid syndrome

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

Page 10: Antiphospholipid syndrome

PATHOPHYSIOLOGY

Page 11: Antiphospholipid syndrome

DIAGNOSIS

Page 12: Antiphospholipid syndrome

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

Page 13: Antiphospholipid syndrome

TREATMENT

Page 14: Antiphospholipid syndrome

RECOMENDATIONS IN PREGNANCY

Page 15: Antiphospholipid syndrome

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.

Page 16: Antiphospholipid syndrome