Catastrophic cerebral antiphospholipid syndrome presenting as cerebral infarction with haemorrhagic transformation after sudden withdrawal of warfarin in a patient with primary antiphospholipid syndrome

Abstract

Catastrophic antiphospholipid syndrome (APS) is caused by thrombotic vascular occlusions that affect both small and large vessels, producing ischaemia in the affected organs. The "catastrophic" variant of the antiphospholipid syndrome (cAPS) develops over a short period of time. Although patients with cAPS represent <1% of all patients with APS, they are usually life threatening with a 50% mortality rate. A strong association with concomitant infection is thought to act as the main trigger of microthromboses in cAPS. Several theories have been proposed to explain these physiopathological features. Some of them suggest the possibility of molecular mimicry between components of infectious microorganisms and natural anticoagulants, which might be involved in the production of cross-reacting antiphospholipid antibodies. We present a case of catastrophic cerebral APS characterised by massive temporal lobe infarction and subsequent haemorrhagic transformation after sudden withdrawal of warfarin.

Antiphospholipid syndrome in renal transplantation

“…Environmental triggers are identified in around half of CAPS patients, and several factors present at the time of transplantation may trigger APS‐related allograft TMA. In this patient, TMA both in the native kidneys and post‐transplantation followed cessation of warfarin, consistent with reports in CAPS . Abrupt withdrawal of warfarin in such patients can increase synthesis of fibrin and thrombin with transient rebound hypercoagulability .…”

“…In this patient, TMA both in the native kidneys and posttransplantation followed cessation of warfarin, consistent with reports in CAPS. 8,58,59 Abrupt withdrawal of warfarin in such patients can increase synthesis of fibrin and thrombin with transient rebound hypercoagulability. 58 Endothelial activation due to surgery is another major precipitant of TMA, reported as second only to infection in triggering CAPS.…”

“…8,58,59 Abrupt withdrawal of warfarin in such patients can increase synthesis of fibrin and thrombin with transient rebound hypercoagulability. 58 Endothelial activation due to surgery is another major precipitant of TMA, reported as second only to infection in triggering CAPS. 8 Thus the combination of surgery, transplant ischaemia-reperfusion injury, alloimmunity and exposure to CNI may all have contributed to endothelial activation and concomitant activation of complement and coagulation, culminating in TMA.…”

RETRACTED: Evaluation of Antiphospholipid Antibodies in Youths Suffering from Cerebral Ischemia

“…Stroke in this age group constitutes 3% of total strokes in America, 15% in Europe, and a recent study estimated its annual prevalence to be 23 in every 100,000 persons [2]. Diagnosis, treatment, and prevention from stroke in this age group is of special importance.…”

Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review

“…Antiphospholipid antibody syndrome (APLS) is a systemic, autoantibody‐mediated disease characterized by venous and/or arterial thrombosis, obstetric complications [ 1 ], and persistently detectable serum titers of antiphospholipid (aPL) antibodies (specifically anti‐β 2 glycoprotein 1, anticardiolipin, and/or lupus anticoagulant antibodies) [ 2 , 3 , 4 ]. In less than 1% of patients with APLS, a life‐threatening complication known as catastrophic aPL antibody syndrome (CAPS) can develop in the presence of precipitating circumstances such as infection [ 5 , 6 ], neoplasm [ 7 ], anticoagulation withdrawal [ 8 ], and pregnancy [ 9 , 10 ]. Although diagnosis can be clinically challenging, international consensus criteria exist specifying diagnosis based on thrombosis of at least three different organs systems over a period of 1 week, confirmed aPL antibodies, and histopathological evidence of multiple small vessel occlusions [ 11 , 12 ].…”