• Diagnosis of anti-phospholipid ab. syndrome (APS)
  • Clinical Criteria
    • Vascular thrombosis: arterial, venous, or small-vessel thrombosis in any tissue
    • Pregnancy morbidity: Early (3) or late (1) spontaneous abortions OR pre-mature birth due to pre-eclampsia, placental insufficiency 
  • Laboratory Criteria
    • Presence of lupus anticoagulant (LA) in plasma 
    • Elevated levels of anticardiolipin (IgG or IgM)
    • Elevated levels of anti–beta-2 glycoprotein I antibodies (IgG or IgM)
  • Each must be present on 2+ occasions at least 12 weeks apart
  
  

  Posted 09/08/16 09:04:39 AM by Matthew Cummings

• Thrombotic microangiopathies -- often difficult to distinguish 
  • TTP: difficult to distinguish from CAPS, must send ADAMTS13 prior to initiation of PLEX 
  • HUS:  atypical HUS --> excessive activation of complement often 2/2 anti-factor H autoantibodies --> eculizumab
  • CAPS: prior hx APS
  • DIC: hypofibrinogenemia, prolonged INR
  • HELLP: pregnancy, abnormal LFTs
  • HIT: Type 2 --> typically thrombocytopenia 4-10 days after exposure to heparin  products w/ vascular thrombosis, screen w/ PF4 abs.
  
  
  
  

  Posted 09/08/16 09:06:42 AM by Matthew Cummings

• Catastrophic APS
  • Rare, life-threatening form of APS -- widespread intravascular thrombosis resulting in multi-organ ischemia and failure
  • Occurs in <1% patients with APS, but may be initial presentation in up to 50%
  • Multiple small vessel occlusions, acute microangiopathy
    • AKI
    • Myocardial injury/ischemia
    • Stroke/encephalopathy
    • ARDS
    • Multi-organ dysfunction syndrome
  
  
  
  

  Posted 09/08/16 09:07:39 AM by Matthew Cummings

• Catastrophic APS Diagnosis
  1. Evidence of clinical disorder of 3+ organ systems
  2. Development of manifestations within 1 week
  3. Laboratory confirmation of presence of aPL ab.
  4. Tissue confirmation of small-vessel occlusion in 1+ organ or tissue
  Typically will initiate therapy based on clinical diagnosis (1-3), but often will push for tissue biopsy 
  
  
  
  

  Posted 09/08/16 09:12:04 AM by Matthew Cummings

• Principles of APS Management
  • Manage thrombotic events, suppress cytokine cascade
    • IV heparin
    • High-dose corticosteroids
    • Plasma exchange +/- IVIG (remove or mitigate effect of anti-cardiolipin ab.)
  • Identify and treat trigger (often infection)
  • Refractory CAPS: emerging role for ritxumab, eculizimab
  
  
  
  

  Posted 09/08/16 09:13:37 AM by Matthew Cummings

• For further reading...

  Approach to catastrophic thrombotic syndromes (Blood)
  CAPS: international guidelines for diagnosis and management
  Atypical HUS (NEJM Review)
  
  

  Posted 09/08/16 09:41:47 AM by Matthew Cummings

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