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Catastrophic antiphospholipid syndrome (CAPS) is a rare and life threatening event characterized by widespread intravascular thrombosis and multi-organ failure. Rarely is CAPS associated with bleeding diathesis, but has been reported in cases with severe thrombocytopenia and acquired prothrombin inhibitors. APS auto-antibodies are heterogeneous and may undergo post-translational modification during antigen stimulation altering its pathogenicity and thrombotic risk. Sepsis and associated disseminated intravascular coagulation is a known phenomenon where cytokines influence pro-coagulant and anti-coagulant pathways on multiple levels to induce haemostatic chaos.

Demonstrate the role of sepsis in triggering life threatening CAPS, and highlight the management strategies used in this highly complex and fatal disease

Prospective case study illustrating two separate atypical CAPS presentations and the management strategies employed

1^st episode (2015): 54F with long standing 27 years of triple positive APS, pro-thrombotic history with recurrent thrombosis despite optimal anticoagulation. Her pro-thrombotic equilibrium was altered after a respiratory tract infection where she presented with severe headaches. Subsequent investigations demonstrated multiple atraumatic intra-cranial haemorrhages followed by concurrent extensive cerebral venous thrombosis.

2^nd episode (2017): She presented with subdural haemorrhage, preceded by fevers and respiratory symptoms. She then developed pleuritic chest pain and dyspnoea after temporary cessation of anticoagulation for 24 hours. Imaging confirmed multiple pulmonary emboli with areas of infarction. Respiratory symptoms worsened with progressive interstitial ground glass changes on CT consistent with atypical pulmonary infection. Shortly after low therapeutic anti-coagulation she developed acute abdominal pain and hypotension. CT confirmed significant bilateral adrenal haemorrhages.

Management Strategies: (A) Rapid reduction in APS pathogenic auto-antibodies via plasma exchange, B cell depletion therapy and immune modulation. (B) Treatment of underlying infectious trigger. (C) Judicious anticoagulation with anti-Xa monitoring and (D) long term hydroxychloroquine and statin therapy

The two life threatening presentations of CAPS were triggered by an infectious event, supporting the biological concept that anti-phospholipid antibodies can be immune modulated altering his pathogenic capabilities creating haemostatic havoc. There are similarities and a degree of overlap with sepsis and the pathophysiology behind disseminated intravascular coagulopathy. Rapid reduction in the pathogenic auto-antibodies using combination plasma exchange, immune modulation and B cell depletion therapy is effective in this acute setting. Judicious anticoagulation and treatment of the precipitating infection is important in turning off the immune response driving this life-threatening coagulopathy.

CAPS is rare and life threatening, often triggered by an infectious event, trauma or temporary cessation of anticoagulation. It requires prompt recognition and timely commencement of therapy.