Case Presentation: A 44-year-old man with thrombocytopenia and hypertension was admitted with acute left pontine ischemic stroke. One week later, he underwent computed tomography (CT) of abdomen for persistent abdominal pain and fever, revealing multiple emboli involving the pulmonary, mesenteric and splenic artery. Doppler studies revealed right lower extremity deep vein thrombosis (DVT) and arterial thrombosis. Subsequently, a PFO was found on transthoracic echo with bubble study. Anti-coagulation with heparin was initiated promptly. Immunologic work up revealed positive lupus anticoagulant (LAC) testing. Given multi-organ thrombosis in the presence of LAC, the overall presentation was attributed to CAPS. High dose steroids were initiated, however the patient continued to have persistent progression of thrombosis/infarcts and developed acute infarct in left middle cerebral artery territory, left leg DVT, and proximal progression of right leg DVT. He underwent small bowel resection for ischemic bowel, complicated by intra-abdominal hematoma and anastomotic leak. Pathology was consistent with micro thrombi and vascular changes seen in systemic thromboembolic disease. In view of severe presentation and life threatening symptoms, decision was made to proceed with three cycles of plasmapheresis, followed by five cycles of intravenous immunoglobulins (IVIG). No further thrombotic events occurred. He gradually improved clinically and was discharged on warfarin and pulse dose steroid taper. He was referred to congenital heart disease specialist for percutaneous closure of PFO.
Discussion: Catastrophic anti-phospholipid antibody syndrome (CAPS) is a rare and potentially lethal variant of anti-phospholipid antibody syndrome (APS) characterized by disseminated arterial and venous thrombosis leading to multi-organ failure. Most CAPS episodes are known to be precipitated by infection, malignancy or surgical procedures. Presence of intra-cardiac shunt, commonly patent foramen ovale (PFO), in combination with underlying hypercoagulable state may increase the risk of diffuse vascular thrombosis.Although most patients with PFO are asymptomatic, those with coexisting hypercoagulable disorder, such as presence of lupus anticoagulant (LAC), may be predisposed to paradoxical embolism causing cryptogenic strokes, such as in our patient. The exact interaction between PFO and hypercoagulable state is poorly understood. Whereas the co-existence of PFO was concerning, workup for concurrent hypercoagualable disorder was warranted in the setting of ongoing vascular thrombosis. In our patient, the massive arterial and venous thrombus burden was likely a manifestation of CAPS, as it is unlikely to develop recurrent venous and arterial emboli in a short time span from PFO. Diagnosis of CAPS requires involvement of three or more organ thromboses in a short time span, presence of anti-phospholipid antibodies, and biopsy evidence of micro thrombi, which were fulfilled in our case. Our patient presented with refractory CAPS which progressed despite initial anti-coagulation and high dose steroids,hence aggressive treatment with plasmapheresis and IVIG was required for rapid improvement.
Conclusions: CAPS is associated with high morbidity and mortality, hence prompt diagnosis and aggressive treatment measures should be initiated once diagnosis is established. Our case emphasizes the importance of hypercoaguable workup in patients with PFO and paradoxical emboli.