Antiphospholipid Antibodies and Recurrent Thrombotic Events: Persistence and Portfolio.

BACKGROUND: There are very limited prospective data on the significance of persistent antiphospholipid antibodies (aPL) and recurrent thrombo-occlusive events (TOEs). We investigated the prognostic value of (1) 2 newer aPL assays, (2) an aPL portfolio and (3) persistent aPL positivity following stroke. METHODS: A total of 1,770 subjects from the APASS-WARSS study underwent further aPL testing for antibodies to phosphatidylserine (aPS) and anti-ß2-glycoprotein-I (anti-ß2GPI) from stored sera. Follow-up aPL status was also tested in a subset of subjects. Primary analysis was based on time to any TOE (ischemic stroke, myocardial infarction, transient ischemic attack, deep vein thrombosis, pulmonary embolism or systemic arterial occlusion)/death at 2 years. Cox proportional hazard analyses assessed whether aPL independently related to outcome. RESULTS: Persistent anti-ß2GPI decreased the time to TOE/death after adjustment for potential confounders (hazards ratio (HR) 2.86, 95% CI 1.21-6.76, p = 0.017). When persistent anti-ß2GPI was combined with another persistently positive aPL, time to TOE/death was also reduced (HR 3.79, 95% CI 1.18-12.14, p = 0.025). Neither persistent anticardiolipin antibodies nor persistent aPS alone nor a single positive anti-ß2GPI nor aPS was associated with decreased time to TOE/death. No single positive aPL, portfolio of baseline aPL or any persistent aPL increased the rate of TOE/death. CONCLUSIONS: Rates of TOE/death were not influenced by aPL results at baseline or follow-up. Persistent anti-ß2GPI alone, and with persistent second aPL, was independently associated with decreased time to TOE/death. Persistent aPL, an aPL portfolio and newer aPL in ischemic stroke patients are not helpful in predicting an increased rate of recurrent TOEs.

Lateral medullary stroke in patient with granulomatous polyangiitis.

Granulomatous polyangiitis (GPA), also known as Wegener granulomatosis, is a systemic antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis that infrequently affects the central nervous system. We report a 41-year-old man with lateral medullary infarction who developed rapidly progressive renal failure. He was diagnosed with GPA based on positive serum c-ANCA and antiproteinase 3 antibodies and demonstration of pauci-immune crescentic glomerulonephritis on kidney biopsy. He was treated with Coumadin, pulse steroids, cyclophosphamide, and plasmapheresis. He had resolution of his neurologic deficits and improvement in renal function. This case report highlights the importance to consider GPA vasculitis in the differential diagnosis of stroke in patients with development of acute kidney injury.

Intravascular lymphomatosis of the brain in a patient with myelodysplastic syndrome.

BACKGROUND: A 77-year-old retired research pharmacologist with a long-standing history of anemia and a recent pathologically confirmed diagnosis of myelodysplastic syndrome was referred to a stroke unit for evaluation of slowly progressive cognitive deterioration, confusion and paroxysmal stroke-like episodes. A previous neurological work-up had revealed no noteworthy abnormalities except for chronic bilateral caudate infarctions seen on MRI and CT examinations of the brain. INVESTIGATIONS: Physical examination, laboratory testing, brain MRI scanning, EEG, transesophageal echocardiography, cerebral angiography, CT scanning, and brain biopsy. DIAGNOSIS: Intravascular lymphomatosis of the brain. MANAGEMENT: Combined chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) and rituximab.