CNS manifestations in acute and chronic graft-versus-host disease

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...

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Autores: Lambert, N, Forte, F, El Moussaoui, M, Monseur, J, Raus, N, Polushin, A, Michonneau, D, Shultz, C, Hogan, WJ, Balaguer-Roselló, A, Gil-Perotín, S, Brijs, J, Chauvet, P, Gavriilaki, M, Carre, M, Dulamea, AO, Chalandon, Y, Salmenniemi, U, Duminuco, A, Ram, R, García-Cadenas, I, Porto, G, Nguyen, S, Smallbone, P, González-Vicent, M, Santoro, JD, Willems, E, Baron, F, Servais, S, Beguin, Y, Maquet, P, CNS GvHD Study Grp
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2025
País:España
Institución:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
Repositorio:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
OAI Identifier:oai:iibsantpau.fundanetsuite.com:p19550
Acceso en línea:https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=19550
Access Level:acceso abierto
Palabra clave:GvHD
neurological complications
encephalitis
brain lesions
spinal cord lesions
immune-mediated
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spelling CNS manifestations in acute and chronic graft-versus-host diseaseLambert, NForte, FEl Moussaoui, MMonseur, JRaus, NPolushin, AMichonneau, DShultz, CHogan, WJBalaguer-Roselló, AGil-Perotín, SBrijs, JChauvet, PGavriilaki, MCarre, MDulamea, AOChalandon, YSalmenniemi, UDuminuco, ARam, RGarcía-Cadenas, IPorto, GNguyen, SSmallbone, PGonzález-Vicent, MSantoro, JDWillems, EBaron, FServais, SBeguin, YMaquet, PCNS GvHD Study GrpGvHDneurological complicationsencephalitisbrain lesionsspinal cord lesionsimmune-mediatedDespite 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 range25-75 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/mu l). 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. In a retrospective international multicentre study, Lambert et al. describe the clinical, biological, radiological, and histopathological characteristics, as well as the course and treatment response, of a large cohort of patients with CNS involvement in graft-versus-host disease.OXFORD UNIV PRESS2025info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=19550BRAINISSN: 00068950ISSNe: 14602156reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pauinstname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)Inglésinfo:eu-repo/semantics/openAccessoai:iibsantpau.fundanetsuite.com:p195502026-06-14T12:41:47Z
dc.title.none.fl_str_mv CNS manifestations in acute and chronic graft-versus-host disease
title CNS manifestations in acute and chronic graft-versus-host disease
spellingShingle CNS manifestations in acute and chronic graft-versus-host disease
Lambert, N
GvHD
neurological complications
encephalitis
brain lesions
spinal cord lesions
immune-mediated
title_short CNS manifestations in acute and chronic graft-versus-host disease
title_full CNS manifestations in acute and chronic graft-versus-host disease
title_fullStr CNS manifestations in acute and chronic graft-versus-host disease
title_full_unstemmed CNS manifestations in acute and chronic graft-versus-host disease
title_sort CNS manifestations in acute and chronic graft-versus-host disease
dc.creator.none.fl_str_mv Lambert, N
Forte, F
El Moussaoui, M
Monseur, J
Raus, N
Polushin, A
Michonneau, D
Shultz, C
Hogan, WJ
Balaguer-Roselló, A
Gil-Perotín, S
Brijs, J
Chauvet, P
Gavriilaki, M
Carre, M
Dulamea, AO
Chalandon, Y
Salmenniemi, U
Duminuco, A
Ram, R
García-Cadenas, I
Porto, G
Nguyen, S
Smallbone, P
González-Vicent, M
Santoro, JD
Willems, E
Baron, F
Servais, S
Beguin, Y
Maquet, P
CNS GvHD Study Grp
author Lambert, N
author_facet Lambert, N
Forte, F
El Moussaoui, M
Monseur, J
Raus, N
Polushin, A
Michonneau, D
Shultz, C
Hogan, WJ
Balaguer-Roselló, A
Gil-Perotín, S
Brijs, J
Chauvet, P
Gavriilaki, M
Carre, M
Dulamea, AO
Chalandon, Y
Salmenniemi, U
Duminuco, A
Ram, R
García-Cadenas, I
Porto, G
Nguyen, S
Smallbone, P
González-Vicent, M
Santoro, JD
Willems, E
Baron, F
Servais, S
Beguin, Y
Maquet, P
CNS GvHD Study Grp
author_role author
author2 Forte, F
El Moussaoui, M
Monseur, J
Raus, N
Polushin, A
Michonneau, D
Shultz, C
Hogan, WJ
Balaguer-Roselló, A
Gil-Perotín, S
Brijs, J
Chauvet, P
Gavriilaki, M
Carre, M
Dulamea, AO
Chalandon, Y
Salmenniemi, U
Duminuco, A
Ram, R
García-Cadenas, I
Porto, G
Nguyen, S
Smallbone, P
González-Vicent, M
Santoro, JD
Willems, E
Baron, F
Servais, S
Beguin, Y
Maquet, P
CNS GvHD Study Grp
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv GvHD
neurological complications
encephalitis
brain lesions
spinal cord lesions
immune-mediated
topic GvHD
neurological complications
encephalitis
brain lesions
spinal cord lesions
immune-mediated
description 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 range25-75 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/mu l). 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. In a retrospective international multicentre study, Lambert et al. describe the clinical, biological, radiological, and histopathological characteristics, as well as the course and treatment response, of a large cohort of patients with CNS involvement in graft-versus-host disease.
publishDate 2025
dc.date.none.fl_str_mv 2025
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=19550
url https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=19550
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv OXFORD UNIV PRESS
publisher.none.fl_str_mv OXFORD UNIV PRESS
dc.source.none.fl_str_mv BRAIN
ISSN: 00068950
ISSNe: 14602156
reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
instname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
instname_str Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
reponame_str r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
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