Cerebral angiitis in four patients with chronic GVHD

• Published in: Bone marrow transplantation (Basingstoke) Vol. 45; no. 7; pp. 1181 - 1188
• Main Authors: Sostak, P, Padovan, C S, Eigenbrod, S, Roeber, S, Segerer, S, Schankin, C, Siegert, S, Saam, T, Theil, D, Kolb, H-J, Kretzschmar, H, Straube, A
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 01.07.2010; Nature Publishing Group
• Subjects: Adult; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Atrophy; Biological and medical sciences; Bone marrow; Bone Marrow Transplantation - adverse effects; Bone marrow, stem cells transplantation. Graft versus host reaction; Brain; Care and treatment; CCR5 protein; CD11a antigen; CD11a Antigen - analysis; Cell Biology; Cell Movement - immunology; Central nervous system; Chemokine receptors; Chronic Disease; Cognitive ability; Complications and side effects; Diagnosis; Diagnosis, Differential; Differential diagnosis; Female; Graft versus host reaction; Graft vs Host Disease - complications; Graft vs Host Disease - diagnosis; Health aspects; Hematology; Humans; Immunofluorescence; Immunohistochemistry; Immunosuppression; Immunosuppressive agents; Immunosuppressive Agents - pharmacology; Internal Medicine; Ischemia; Leukoencephalopathy; Longitudinal Studies; Magnetic Resonance Imaging; Male; Medical sciences; Medicine; Medicine & Public Health; Neuroimaging; Neurological diseases; Neurology; Organs; original-article; Public Health; Receptors; Receptors, CCR5 - analysis; Risk factors; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Stem cell transplantation; Stem Cells; Survivors; Transfusions. Complications. Transfusion reactions. Cell and gene therapy; Transplantation; Transplantation, Homologous; Vascular diseases and vascular malformations of the nervous system; Vasculitis; Vasculitis, Central Nervous System - diagnosis; Vasculitis, Central Nervous System - etiology; Vasculitis, Central Nervous System - pathology; Young Adult; Germany; cerebral angiitis; chronic GVHD; neurological complications; cerebrovascular disease; Human; Immunopathology; Graft versus host disease; Nervous system diseases; Hematology; Stem cell; Central nervous system; Hematopoietic cell; Cardiovascular disease; Cerebral disorder; Encephalon; Vascular disease; Chronic; Vasculitis; Central nervous system disease; Cerebrovascular disease
• ISSN: 0268-3369; 1476-5365
• DOI: 10.1038/bmt.2009.323

There is growing evidence that GVHD affects the central nervous system (CNS). In this study, we describe the long-term follow-up of four allogeneic BM recipients who developed cerebral angiitis-like disease probably due to GVHD. The patients developed focal neurological signs, cognitive deficits and/or coma in association with GVHD, 2–18 years after transplantation, following reduction of immunosuppressive therapy. Magnetic resonance imaging was variable, showing generalized brain atrophy, ischemic lesions or leukoencephalopathy. Diagnosis of cerebral angiitis was confirmed by histopathological analysis of bioptic brain tissue and response to immunosuppressive therapy. By means of immunohistochemistry and immunofluorescence, perivascular lymphomononuclear cerebral infiltrates were shown to express the adhesion receptor, CD11a, and the chemokine receptor, CCR5. Our findings imply that GVHD should be considered in the differential diagnosis of noninfectious angiitis-like disease of the CNS in long-term survivors after allogeneic BMT. Infiltrating cells, in analogy to typical target organs of GVHD such as skin or liver, expressed CD11a and CCR5. These findings could be of etiopathological, diagnostic and therapeutic relevance.