Chronic-graft-versus-host-disease-related polymyositis: a 17-months-old child with a rare and late complication of haematopoietic stem cell transplantation.

Background: Chronic graft versus host disease (cGVHD) occurs in 20-30% of paediatric patients receiving haemopoietic stem cell transplantation (HSCT). Neuromuscular disorders such as polymyositis are considered a rare and distinctive but non-diagnostic manifestation of cGVHD and, in absence of other...

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Main Authors: Matteo Chinello, Rita Balter, Massimiliano De Bortoli, Virginia Vitale, Ada Zaccaron, Elisa Bonetti, Paola Tonin, Gaetano Vattemi, Valeria Guglielmi, Simone Cesaro
Format: Article
Language:English
Published: PAGEPress Publications 2020-01-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
Online Access:http://mjhid.org/index.php/mjhid/article/view/4029
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author Matteo Chinello
Rita Balter
Massimiliano De Bortoli
Virginia Vitale
Ada Zaccaron
Elisa Bonetti
Paola Tonin
Gaetano Vattemi
Valeria Guglielmi
Simone Cesaro
author_facet Matteo Chinello
Rita Balter
Massimiliano De Bortoli
Virginia Vitale
Ada Zaccaron
Elisa Bonetti
Paola Tonin
Gaetano Vattemi
Valeria Guglielmi
Simone Cesaro
author_sort Matteo Chinello
collection DOAJ
description Background: Chronic graft versus host disease (cGVHD) occurs in 20-30% of paediatric patients receiving haemopoietic stem cell transplantation (HSCT). Neuromuscular disorders such as polymyositis are considered a rare and distinctive but non-diagnostic manifestation of cGVHD and, in absence of other characteristic signs and symptoms, biopsy is highly recommended to exclude other causes. Case report: We report a case of a 17-months-old child affected by hemophagocytic lymphohistiocytosis who underwent a matched unrelated donor haematopoietic stem cell transplantation (HSCT). She developed a severe cGVHD-related polymyositis that was successfully treated with high-dose steroid therapy, rituximab and sirolimus. Conclusions: This is the first case of cGVHD-related-polymyositis described in a pediatric patient which was successfully treated with rituximab.
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institution Kabale University
issn 2035-3006
language English
publishDate 2020-01-01
publisher PAGEPress Publications
record_format Article
series Mediterranean Journal of Hematology and Infectious Diseases
spelling doaj-art-9efb42b6a82c40ad832b6584ceb59da02024-12-02T00:01:15ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062020-01-0112110.4084/mjhid.2020.002Chronic-graft-versus-host-disease-related polymyositis: a 17-months-old child with a rare and late complication of haematopoietic stem cell transplantation.Matteo ChinelloRita BalterMassimiliano De BortoliVirginia VitaleAda ZaccaronElisa BonettiPaola ToninGaetano VattemiValeria GuglielmiSimone CesaroBackground: Chronic graft versus host disease (cGVHD) occurs in 20-30% of paediatric patients receiving haemopoietic stem cell transplantation (HSCT). Neuromuscular disorders such as polymyositis are considered a rare and distinctive but non-diagnostic manifestation of cGVHD and, in absence of other characteristic signs and symptoms, biopsy is highly recommended to exclude other causes. Case report: We report a case of a 17-months-old child affected by hemophagocytic lymphohistiocytosis who underwent a matched unrelated donor haematopoietic stem cell transplantation (HSCT). She developed a severe cGVHD-related polymyositis that was successfully treated with high-dose steroid therapy, rituximab and sirolimus. Conclusions: This is the first case of cGVHD-related-polymyositis described in a pediatric patient which was successfully treated with rituximab.http://mjhid.org/index.php/mjhid/article/view/4029
spellingShingle Matteo Chinello
Rita Balter
Massimiliano De Bortoli
Virginia Vitale
Ada Zaccaron
Elisa Bonetti
Paola Tonin
Gaetano Vattemi
Valeria Guglielmi
Simone Cesaro
Chronic-graft-versus-host-disease-related polymyositis: a 17-months-old child with a rare and late complication of haematopoietic stem cell transplantation.
Mediterranean Journal of Hematology and Infectious Diseases
title Chronic-graft-versus-host-disease-related polymyositis: a 17-months-old child with a rare and late complication of haematopoietic stem cell transplantation.
title_full Chronic-graft-versus-host-disease-related polymyositis: a 17-months-old child with a rare and late complication of haematopoietic stem cell transplantation.
title_fullStr Chronic-graft-versus-host-disease-related polymyositis: a 17-months-old child with a rare and late complication of haematopoietic stem cell transplantation.
title_full_unstemmed Chronic-graft-versus-host-disease-related polymyositis: a 17-months-old child with a rare and late complication of haematopoietic stem cell transplantation.
title_short Chronic-graft-versus-host-disease-related polymyositis: a 17-months-old child with a rare and late complication of haematopoietic stem cell transplantation.
title_sort chronic graft versus host disease related polymyositis a 17 months old child with a rare and late complication of haematopoietic stem cell transplantation
url http://mjhid.org/index.php/mjhid/article/view/4029
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