Rapidly Progressive Occlusive Intracranial Vasculopathy in Graft-versus-Host-Disease (P1.299)

Objective: Describe a novel mechanism for strokes in a patient with active graft versus host disease. Background: Cerebrovascular complications occur in ~3% of allogenic hematopoietic stem cell transplants (HSCT). The CNS is not traditionally affected by graft versus host disease (GVHD) but rare cases with pathologic confirmation report perivascular infiltrates cause leukoencephalopathy, encephalitis or vasculitis with subcortical ischemia or hemorrhage. Design/Methods: Case Report Results: Five years after allogenic HSCT for B cell chronic lymphocytic leukemia complicated by sclerodermoid GVHD, a 54 year old diabetic man received IVtPA for acute left hemiplegia with full recovery. Recurrent TIAs of ataxic hemiparesis for 2 months failed aspirin, clopidogrel, heparin and ticagrelor then improved with tacrolimus. MRA and CTA were normal but TCD was consistent with RMCA stenosis >50%. At 9–11 months, recurrent right and new left MCA TIAs accompanied progressive sclerodermoid GVHD. Angiography revealed right MCA occlusion, left MCA and multifocal intracranial stenoses interpreted as vasculitis but not confirmed by LP or brain biopsy. Headaches temporarily improved with high-dose steroids but despite cyclophosphamide, he suffered recurrent right PCA and MCA infarcts and died 2 months later from bacterial meningitis. Neuropathology revealed adventitial neutrophils from meningitis but no medial or endothelial inflammation to suggest vasculitis. The MCAs were occluded by prominent intimal hyperplasia with collagen deposition and medial disruption but no atherosclerotic plaque. Minimal atherosclerosis was found in the coronary arteries. Conclusions: To our knowledge, this is the first case of pathologically confirmed chronic GVHD involving the intracranial vasculature due to a rapidly progressive non-inflammatory occlusive vasculopathy. The poor response to multiple therapies and pathology is akin to allograft vasculopathy. Vasculitis should not be assumed to be the cause of multifocal angiographic stenoses and future cases should consider palliative intracranial angioplasty or stenting. Disclosure: Dr. Bowen has nothing to disclose. Dr. Silver has nothing to disclose. Dr. Sila has received personal compensation for activities with Axio Research, Hoffman La Roche, Medtronic, Janssen, and Daiichi-Sanyko. Dr. Sila has received personal compensation in an editorial capacity for Current Cardiology Reports and UpToDate. Dr. Sila has received research support from AGA Medical and Athersys.