Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood
- Autores
- Aricò, Maurizio; Astigarraga, Itziar; Braier, Jorge; Donadieu, Jean; Gadner, Helmut; Glogova, Evgenia; Grois, Nicole; Henter, Jan-Inge; Janka, Gritta; McClain, Kenneth L.; Ladisch, Stephan; Pötschger, Ulrike; Rosso, Diego; Thiem, Elfriede; Weitzman, Sheila; Windebank, Kevin; Minkov, Milen; For the Histiocyte Society
- Año de publicación
- 2014
- Idioma
- inglés
- Tipo de recurso
- artículo
- Estado
- versión publicada
- Descripción
- Skeletal involvement is generally, but not universally, characteristic of Langerhans cell histiocytosis (LCH). We investigated whether the presence of bone lesions at diagnosis is a prognostic factor for survival in LCH. Nine hundred and thirty-eight children with multisystem (MS) LCH, both high (386 RO+) and low (RO−) risk, were evaluated for bone lesions at diagnosis. Risk organ (RO+) involvement was defined as: haematopoietic system (haemoglobin <100 g/l, and/or white blood cell count <4·0 × 109/l and/or platelet count <100 × 109/l), spleen (>2 cm below the costal margin), liver (>3 cm and/or hypoproteinaemia, hypoalbuminaemia, hyperbilirubinaemia, and/or increased aspartate transaminase/alanine transaminase). Given the general view that prognosis in LCH worsens with increasing extent of disease, the surprising finding was that in MS+RO+ LCH the probability of survival with bone involvement 74 ± 3% (n = 230, 56 events) was reduced to 62 ± 4% (n = 156, 55 events) if this was absent (P = 0·007). An even greater difference was seen in the subgroup of patients with both liver and either haematopoiesis or spleen involvement: 61 ± 5% survival (n = 105; 52 events) if patients had bony lesions, versus 47 ± 5% (n = 111; 39 events) if they did not (P = 0·014). This difference was retained in multivariate analysis (P = 0·048). Although as yet unexplained, we conclude that bone involvement at diagnosis is a previously unrecognized favourable prognostic factor in MS+RO+ LCH.
Fil: Aricò, Maurizio. Azienda Sanitaria Provinciale; Italia
Fil: Astigarraga, Itziar. Bio Cruces Health Research Institute; España. Universidad del País Vasco; España
Fil: Braier, Jorge. Gobierno de la Ciudad de Buenos Aires. Hospital de Pediatría "Juan P. Garrahan"; Argentina
Fil: Donadieu, Jean. Hospital Trousseau; Francia
Fil: Gadner, Helmut. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; Austria
Fil: Glogova, Evgenia. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; Austria
Fil: Grois, Nicole. Children's Cancer Research Institute; Austria. St. Anna Children's Hospital; Austria
Fil: Henter, Jan-Inge. Karolinska Huddinge Hospital. Karolinska Institutet; Suecia
Fil: Janka, Gritta. University Medical Centre. Department of Haematology and Oncology; Alemania
Fil: McClain, Kenneth L.. Texas Children's Cancer and Hematology Centers ; Estados Unidos
Fil: Ladisch, Stephan. Children's National Medical Center ; Estados Unidos
Fil: Pötschger, Ulrike. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; Austria
Fil: Rosso, Diego. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños Pedro Elizalde (ex Casa Cuna); Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Thiem, Elfriede. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; Austria
Fil: Weitzman, Sheila. Hospital for Sick Children. Division of Hematology/Oncology; Canadá
Fil: Windebank, Kevin. University of Newcastle; Reino Unido
Fil: Minkov, Milen. Children's Cancer Research Institute; Austria. St. Anna Children's Hospital; Austria
Fil: For the Histiocyte Society. - Materia
-
Histiocytosis
Childhood
Bone Involvement
Prognostic Factor
Langerhans Cell Histiocytosis
Multsystem Disease
Bone Lesion
Risck Organ - Nivel de accesibilidad
- acceso abierto
- Condiciones de uso
- https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
- Repositorio
.jpg)
- Institución
- Consejo Nacional de Investigaciones Científicas y Técnicas
- OAI Identificador
- oai:ri.conicet.gov.ar:11336/15796
Ver los metadatos del registro completo
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Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhoodAricò, MaurizioAstigarraga, ItziarBraier, JorgeDonadieu, JeanGadner, HelmutGlogova, EvgeniaGrois, NicoleHenter, Jan-IngeJanka, GrittaMcClain, Kenneth L.Ladisch, StephanPötschger, UlrikeRosso, DiegoThiem, ElfriedeWeitzman, SheilaWindebank, KevinMinkov, MilenFor the Histiocyte SocietyHistiocytosisChildhoodBone InvolvementPrognostic FactorLangerhans Cell HistiocytosisMultsystem DiseaseBone LesionRisck Organhttps://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3Skeletal involvement is generally, but not universally, characteristic of Langerhans cell histiocytosis (LCH). We investigated whether the presence of bone lesions at diagnosis is a prognostic factor for survival in LCH. Nine hundred and thirty-eight children with multisystem (MS) LCH, both high (386 RO+) and low (RO−) risk, were evaluated for bone lesions at diagnosis. Risk organ (RO+) involvement was defined as: haematopoietic system (haemoglobin <100 g/l, and/or white blood cell count <4·0 × 109/l and/or platelet count <100 × 109/l), spleen (>2 cm below the costal margin), liver (>3 cm and/or hypoproteinaemia, hypoalbuminaemia, hyperbilirubinaemia, and/or increased aspartate transaminase/alanine transaminase). Given the general view that prognosis in LCH worsens with increasing extent of disease, the surprising finding was that in MS+RO+ LCH the probability of survival with bone involvement 74 ± 3% (n = 230, 56 events) was reduced to 62 ± 4% (n = 156, 55 events) if this was absent (P = 0·007). An even greater difference was seen in the subgroup of patients with both liver and either haematopoiesis or spleen involvement: 61 ± 5% survival (n = 105; 52 events) if patients had bony lesions, versus 47 ± 5% (n = 111; 39 events) if they did not (P = 0·014). This difference was retained in multivariate analysis (P = 0·048). Although as yet unexplained, we conclude that bone involvement at diagnosis is a previously unrecognized favourable prognostic factor in MS+RO+ LCH.Fil: Aricò, Maurizio. Azienda Sanitaria Provinciale; ItaliaFil: Astigarraga, Itziar. Bio Cruces Health Research Institute; España. Universidad del País Vasco; EspañaFil: Braier, Jorge. Gobierno de la Ciudad de Buenos Aires. Hospital de Pediatría "Juan P. Garrahan"; ArgentinaFil: Donadieu, Jean. Hospital Trousseau; FranciaFil: Gadner, Helmut. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; AustriaFil: Glogova, Evgenia. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; AustriaFil: Grois, Nicole. Children's Cancer Research Institute; Austria. St. Anna Children's Hospital; AustriaFil: Henter, Jan-Inge. Karolinska Huddinge Hospital. Karolinska Institutet; SueciaFil: Janka, Gritta. University Medical Centre. Department of Haematology and Oncology; AlemaniaFil: McClain, Kenneth L.. Texas Children's Cancer and Hematology Centers ; Estados UnidosFil: Ladisch, Stephan. Children's National Medical Center ; Estados UnidosFil: Pötschger, Ulrike. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; AustriaFil: Rosso, Diego. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños Pedro Elizalde (ex Casa Cuna); Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Thiem, Elfriede. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; AustriaFil: Weitzman, Sheila. Hospital for Sick Children. Division of Hematology/Oncology; CanadáFil: Windebank, Kevin. University of Newcastle; Reino UnidoFil: Minkov, Milen. Children's Cancer Research Institute; Austria. St. Anna Children's Hospital; AustriaFil: For the Histiocyte Society.Wiley2014-12info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/vnd.openxmlformats-officedocument.wordprocessingml.documentapplication/pdfhttp://hdl.handle.net/11336/15796Aricò, Maurizio; Astigarraga, Itziar; Braier, Jorge; Donadieu, Jean; Gadner, Helmut; et al.; Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood; Wiley; British Journal Of Haematology; 169; 2; 12-2014; 241-2480007-10481365-2141enginfo:eu-repo/semantics/altIdentifier/doi/10.1111/bjh.13271info:eu-repo/semantics/altIdentifier/url/http://onlinelibrary.wiley.com/doi/10.1111/bjh.13271/fullinfo:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-03T09:52:06Zoai:ri.conicet.gov.ar:11336/15796instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-03 09:52:06.672CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse |
| dc.title.none.fl_str_mv |
Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood |
| title |
Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood |
| spellingShingle |
Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood Aricò, Maurizio Histiocytosis Childhood Bone Involvement Prognostic Factor Langerhans Cell Histiocytosis Multsystem Disease Bone Lesion Risck Organ |
| title_short |
Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood |
| title_full |
Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood |
| title_fullStr |
Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood |
| title_full_unstemmed |
Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood |
| title_sort |
Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood |
| dc.creator.none.fl_str_mv |
Aricò, Maurizio Astigarraga, Itziar Braier, Jorge Donadieu, Jean Gadner, Helmut Glogova, Evgenia Grois, Nicole Henter, Jan-Inge Janka, Gritta McClain, Kenneth L. Ladisch, Stephan Pötschger, Ulrike Rosso, Diego Thiem, Elfriede Weitzman, Sheila Windebank, Kevin Minkov, Milen For the Histiocyte Society |
| author |
Aricò, Maurizio |
| author_facet |
Aricò, Maurizio Astigarraga, Itziar Braier, Jorge Donadieu, Jean Gadner, Helmut Glogova, Evgenia Grois, Nicole Henter, Jan-Inge Janka, Gritta McClain, Kenneth L. Ladisch, Stephan Pötschger, Ulrike Rosso, Diego Thiem, Elfriede Weitzman, Sheila Windebank, Kevin Minkov, Milen For the Histiocyte Society |
| author_role |
author |
| author2 |
Astigarraga, Itziar Braier, Jorge Donadieu, Jean Gadner, Helmut Glogova, Evgenia Grois, Nicole Henter, Jan-Inge Janka, Gritta McClain, Kenneth L. Ladisch, Stephan Pötschger, Ulrike Rosso, Diego Thiem, Elfriede Weitzman, Sheila Windebank, Kevin Minkov, Milen For the Histiocyte Society |
| author2_role |
author author author author author author author author author author author author author author author author author |
| dc.subject.none.fl_str_mv |
Histiocytosis Childhood Bone Involvement Prognostic Factor Langerhans Cell Histiocytosis Multsystem Disease Bone Lesion Risck Organ |
| topic |
Histiocytosis Childhood Bone Involvement Prognostic Factor Langerhans Cell Histiocytosis Multsystem Disease Bone Lesion Risck Organ |
| purl_subject.fl_str_mv |
https://purl.org/becyt/ford/3.2 https://purl.org/becyt/ford/3 |
| dc.description.none.fl_txt_mv |
Skeletal involvement is generally, but not universally, characteristic of Langerhans cell histiocytosis (LCH). We investigated whether the presence of bone lesions at diagnosis is a prognostic factor for survival in LCH. Nine hundred and thirty-eight children with multisystem (MS) LCH, both high (386 RO+) and low (RO−) risk, were evaluated for bone lesions at diagnosis. Risk organ (RO+) involvement was defined as: haematopoietic system (haemoglobin <100 g/l, and/or white blood cell count <4·0 × 109/l and/or platelet count <100 × 109/l), spleen (>2 cm below the costal margin), liver (>3 cm and/or hypoproteinaemia, hypoalbuminaemia, hyperbilirubinaemia, and/or increased aspartate transaminase/alanine transaminase). Given the general view that prognosis in LCH worsens with increasing extent of disease, the surprising finding was that in MS+RO+ LCH the probability of survival with bone involvement 74 ± 3% (n = 230, 56 events) was reduced to 62 ± 4% (n = 156, 55 events) if this was absent (P = 0·007). An even greater difference was seen in the subgroup of patients with both liver and either haematopoiesis or spleen involvement: 61 ± 5% survival (n = 105; 52 events) if patients had bony lesions, versus 47 ± 5% (n = 111; 39 events) if they did not (P = 0·014). This difference was retained in multivariate analysis (P = 0·048). Although as yet unexplained, we conclude that bone involvement at diagnosis is a previously unrecognized favourable prognostic factor in MS+RO+ LCH. Fil: Aricò, Maurizio. Azienda Sanitaria Provinciale; Italia Fil: Astigarraga, Itziar. Bio Cruces Health Research Institute; España. Universidad del País Vasco; España Fil: Braier, Jorge. Gobierno de la Ciudad de Buenos Aires. Hospital de Pediatría "Juan P. Garrahan"; Argentina Fil: Donadieu, Jean. Hospital Trousseau; Francia Fil: Gadner, Helmut. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; Austria Fil: Glogova, Evgenia. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; Austria Fil: Grois, Nicole. Children's Cancer Research Institute; Austria. St. Anna Children's Hospital; Austria Fil: Henter, Jan-Inge. Karolinska Huddinge Hospital. Karolinska Institutet; Suecia Fil: Janka, Gritta. University Medical Centre. Department of Haematology and Oncology; Alemania Fil: McClain, Kenneth L.. Texas Children's Cancer and Hematology Centers ; Estados Unidos Fil: Ladisch, Stephan. Children's National Medical Center ; Estados Unidos Fil: Pötschger, Ulrike. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; Austria Fil: Rosso, Diego. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños Pedro Elizalde (ex Casa Cuna); Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina Fil: Thiem, Elfriede. St. Anna Children's Hospital; Austria. Children's Cancer Research Institute; Austria Fil: Weitzman, Sheila. Hospital for Sick Children. Division of Hematology/Oncology; Canadá Fil: Windebank, Kevin. University of Newcastle; Reino Unido Fil: Minkov, Milen. Children's Cancer Research Institute; Austria. St. Anna Children's Hospital; Austria Fil: For the Histiocyte Society. |
| description |
Skeletal involvement is generally, but not universally, characteristic of Langerhans cell histiocytosis (LCH). We investigated whether the presence of bone lesions at diagnosis is a prognostic factor for survival in LCH. Nine hundred and thirty-eight children with multisystem (MS) LCH, both high (386 RO+) and low (RO−) risk, were evaluated for bone lesions at diagnosis. Risk organ (RO+) involvement was defined as: haematopoietic system (haemoglobin <100 g/l, and/or white blood cell count <4·0 × 109/l and/or platelet count <100 × 109/l), spleen (>2 cm below the costal margin), liver (>3 cm and/or hypoproteinaemia, hypoalbuminaemia, hyperbilirubinaemia, and/or increased aspartate transaminase/alanine transaminase). Given the general view that prognosis in LCH worsens with increasing extent of disease, the surprising finding was that in MS+RO+ LCH the probability of survival with bone involvement 74 ± 3% (n = 230, 56 events) was reduced to 62 ± 4% (n = 156, 55 events) if this was absent (P = 0·007). An even greater difference was seen in the subgroup of patients with both liver and either haematopoiesis or spleen involvement: 61 ± 5% survival (n = 105; 52 events) if patients had bony lesions, versus 47 ± 5% (n = 111; 39 events) if they did not (P = 0·014). This difference was retained in multivariate analysis (P = 0·048). Although as yet unexplained, we conclude that bone involvement at diagnosis is a previously unrecognized favourable prognostic factor in MS+RO+ LCH. |
| publishDate |
2014 |
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2014-12 |
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http://hdl.handle.net/11336/15796 Aricò, Maurizio; Astigarraga, Itziar; Braier, Jorge; Donadieu, Jean; Gadner, Helmut; et al.; Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood; Wiley; British Journal Of Haematology; 169; 2; 12-2014; 241-248 0007-1048 1365-2141 |
| url |
http://hdl.handle.net/11336/15796 |
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Aricò, Maurizio; Astigarraga, Itziar; Braier, Jorge; Donadieu, Jean; Gadner, Helmut; et al.; Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood; Wiley; British Journal Of Haematology; 169; 2; 12-2014; 241-248 0007-1048 1365-2141 |
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