CNS manifestations in acute and chronic graft-versus-host disease
Lambert, N. (University Hospital of Liège)
Forte, F. (University Hospital of Liège)
Moussaoui, M.E. (University Hospital of Liège)
Monseur, J. (University of Liège)
Raus, N. (Centre hospitalier Lyon sud)
Polushin, A. (First Pavlov State Medical University of St. Peterburg)
Michonneau, D. (Université Paris Cité)
Shultz, C. (Mayo Clinic (Rochester, Estats Units d'Amèrica))
Hogan, W.J. (Mayo Clinic (Rochester, Estats Units d'Amèrica))
Balaguer-Roselló, A. (Hospital Universitari i Politècnic La Fe (València))
Gil-Perotín, S. (Hospital Universitari i Politècnic La Fe (València))
Brijs, J. (University Hospitals Leuven (Bèlgica))
Chauvet, P. (CHU de Lille. Maladies du Sang. Université de Lille)
Gavriilaki, M. (Aristotle University of Thessaloniki)
Carre, M. (Hôpital Michallon)
Dulamea, A.O. (University of Medicine and Pharmacy 'Carol Davila')
Chalandon, Y. (University of Geneva)
Salmenniemi, U. (HUCH Comprehensive Cancer Center)
Duminuco, A. (A.O.U. Policlinico 'G.Rodolico-San Marco')
Ram, R. (Tel Aviv University)
Garcia Cadenas, Irene (Institut de Recerca Sant Pau)
Porto, G. (Grande Ospedale Metropolitano 'Bianchi-Melacrino-Morelli')
Nguyen, S. (Hospital de la Pitié-Salpêtrière (París, França))
Smallbone, P. (Fiona Stanley Hospital)
González-Vicent, M. (Hospital Niño Jesus)
Santoro, J.D. (Children's Hospital Los Angeles (Estats Units d'Amèrica))
Willems, E. (University Hospital of Liège)
Baron, Frederic (University Hospital of Liège)
Servais, S. (University Hospital of Liège)
Beguin, Yves (University Hospital of Liège)
Maquet, P. (University Hospital of Liège)
Universitat Autònoma de Barcelona

Data:	2025
Resum:	Despite the growing evidence supporting the existence of CNS involvement in acute and chronic graft-versus-host disease (CNS-GvHD), the characteristics and course of the disease are still largely unknown. In this multicentre retrospective study, we analysed the clinical, biological, radiological and histopathological characteristics, as well as the clinical course of 66 patients diagnosed with possible CNS-GvHD (pCNS-GvHD), selected by predetermined diagnostic criteria. Results were then contrasted depending on whether pCNS-GvHD onset occurred before or after Day 100 following allogeneic haematopoietic stem cell transplantation (allo-HSCT). The median time between allo-HSCT and pCNS-GvHD onset was 149 days (interquartile range 48-321), and pCNS-GvHD onset occurred before Day 100 following transplantation in 44% of patients. The most frequent findings at presentation were cognitive impairment (41%), paresis (21%), altered consciousness (20%), sensory impairment (18%) and headache (15%). Clinical presentation did not significantly differ between patients with pCNS-GvHD occurring before or after Day 100 following transplantation. Brain MRI found abnormalities compatible with the clinical picture in 57% of patients, while CT detected abnormalities in only 7%. Seven patients had documented spinal cord MRI abnormalities, all of them with pCNS-GvHD occurring after Day 100 following transplantation. In the CSF, the white blood cell count was increased in 56% of the population (median 18 cells/μl). Histopathological analyses were performed on 12 specimens and were suggestive of pCNS-GvHD in 10. All compatible specimens showed parenchymal and perivascular infiltration by CD3+ and CD163+ cells. Immunosuppressive therapy was prescribed in 97% of patients, achieving complete clinical response in 27%, partial improvement in 47% and stable disease in 6%. Response to immunosuppressive therapy did not differ significantly between patients with pCNS-GvHD occurring before or after Day 100 following transplantation. Clinical relapse was observed in 31% of patients who initially responded to treatment. One-year overall survival following pCNS-GvHD onset was 41%. Onset before Day 100 following haematopoietic stem cell transplantation [hazard ratio with 95% confidence interval: 2. 1 (1. 0-4. 5); P = 0. 041] and altered consciousness at initial presentation [3. 0 (1. 3-6. 7); P = 0. 0077] were associated with a reduced 1-year overall survival probability. Among surviving patients, 61% had neurological sequelae. This study supports that immune-mediated CNS manifestations may occur following allo-HSCT. These can be associated with both acute and chronic GvHD and carry a grim prognosis. The clinical presentation as well as the radiological and biological findings appear variable.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Matèria:	GvHD ; Brain lesions ; Encephalitis ; Immune-mediated ; Neurological complications ; Spinal cord lesions
Publicat a:	Brain, Vol. 148 Núm. 4 (january 2025) , p. 1122-1133, ISSN 1460-2156

DOI: 10.1093/brain/awae340
PMID: 39442000

12 p, 712.5 KB

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