Therapeutic Plasma Exchange After Spontaneous Intracranial Hemorrhage for a Patient With Antiphospholipid Syndrome and Lupus Anticoagulant Hypoprothrombinemia.

Antiphospholipid syndrome (APS) is characterized by the presence of antiphospholipid antibodies (aPL), macro- and micro-vascular thromboembolic complications. Lupus anticoagulant-hypoprothrombinemia (LAHPS) may confound the diagnosis and management of bleeding. Catastrophic APS has a category 1 indication for therapeutic plasma exchange (TPE). However, in patients with APS, LAHPS, and intracranial hemorrhage (ICH), TPE is not well described. A 47-year-old man with known APS anticoagulated on warfarin was transferred for diffuse spontaneous subdural hemorrhages (SDH) with somnolence. aPL levels were elevated on presentation; anti-β2-glycoprotein-I antibody (aβ2GPI) IgG was higher than the reportable range. Factor II activity level was 20% despite holding warfarin: concerning for LAHPS. TPE was initiated to minimize risk of thromboembolism while holding anticoagulation. Level of consciousness improved by the second TPE. An acute lacunar infarct was detected on MRI, but this may have occurred before initiating TPE. Measures of lupus anticoagulant and anticardiolipin (aCL) IgG decreased initially, but aβ2GPI IgG remained above the reportable range. Both aCL and aβ2GPI IgM titers increased initially but decreased by day 31. Factor II activity level improved but remained below normal. Serial imaging showed resolution of SDH without new infarction. In patients with APS and recurrent thromboembolic disease, assessment and treatment of ICH may be confounded by LAHPS. Reversal of anticoagulation is reserved for patients in extremis, and treatment of LAHPS has previously been associated with thrombosis. In this context, TPE may be considered in combination with steroids and rituximab to bridge overlapping thromboembolic and hemorrhagic risk.