Cerebral angiitis in four patients with chronic GVHD.
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.
Sostak
P
P
Department of Neurology, Klinikum Grosshadern, Ludwig Maximilian University, Munich, Germany. Petra.Sostak@med.uni-muenchen.de
Padovan
C S
CS
Eigenbrod
S
S
Roeber
S
S
Segerer
S
S
Schankin
C
C
Siegert
S
S
Saam
T
T
Theil
D
D
Kolb
H-J
HJ
Kretzschmar
H
H
Straube
A
A
D000328:Adult; D016026:Bone Marrow Transplantation / Q000009:adverse effects; D040881:CD11a Antigen / Q000032:analysis; D002465:Cell Movement / Q000276:immunology; D002908:Chronic Disease; D003937:Diagnosis, Differential; D005260:Female; D006086:Graft vs Host Disease / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007166:Immunosuppressive Agents / Q000494:pharmacology*; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D019713:Receptors, CCR5 / Q000032:analysis; D017741:Survivors; D014184:Transplantation, Homologous; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000209:etiology* / Q000473:pathology; D055815:Young Adult
D040881:CD11a Antigen; D007166:Immunosuppressive Agents; D019713:Receptors, CCR5