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 the absence of o...

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Detalles Bibliográficos
Autores principales: Chinello, Matteo, Balter, Rita, De Bortoli, Massimiliano, Vitale, Virginia, Zaccaron, Ada, Bonetti, Elisa, Tonin, Paola, Vattemi, Gaetano, Guglielmi, Valeria, Cesaro, Simone
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Università Cattolica del Sacro Cuore 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6951354/
https://www.ncbi.nlm.nih.gov/pubmed/31934312
http://dx.doi.org/10.4084/MJHID.2020.002
Descripción
Sumario: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 the 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 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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