Hematology: Antiphospholipid Syndrome

Definition

• Autoimmune Disease Due to Antiphospholipid Antibodies
• Primarily Occurs in Presence of Underlying Systemic Autoimmune Disease
  • Particularly Systemic Lupus Erythematosus (SLE)

Presentation

• Venous Thromboembolism (VTE) – Most Common Manifestation
  • High Rate of Recurrent VTE: 5-12% Annually
• Thrombocytopenia
• Livedo Reticularis (Netlike Pattern of Reddish-Blue Skin Discoloration)
• Stroke/TIA
• Fetal Loss

Diagnosis – Sapporo/Sydney Criteria

• At Least One Clinical Criteria & At Least One Laboratory Criteria
• Clinical Criteria:
  • VTE or Arterial Thrombosis
  • Loss of Pregnancy After 10 Weeks if Fetus Morphologically Normal
  • Premature Birth of Morphologically Normal Fetus Before 34 Weeks Due to Eclampsia or Placental Insufficiency
  • Three or More Unexplained Consecutive Spontaneous Abortions Before 10 Weeks
• Laboratory Criteria:
  • Lupus Anticoagulant on Two or More Occasions At Least 12 Weeks Apart
  • Anticardiolipin Antibody on Two or More Occasions At Least 12 Weeks Apart
  • Anti-Beta-2 Glycoprotein-I Antibody on Two or More Occasions At Least 12 Weeks Apart

Management

• VTE Treatment: Lifelong Anticoagulation
• VTE Prevention: Prophylactic Anticoagulation is Controversial if There Are No Past Thrombotic Events