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Late differentiation syndrome in acute promyelocytic leukemia: a challenging diagnosis

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Detalhes bibliográficos
Resumo:Detailed knowledge about differentiation syndrome (DS) has remained limited. There are 2 large studies conducted by the Spanish workgroup PETHEMA (Programa Español de Tratamientos en Hematología; Spanish Program on Hematology Treatments) and the European group trial (LPA 96-99 and APL 93) in which the incidence, characteristics, prognostic factors and outcome of patients developing DS are evaluated. Both have described the median time of DS development between 10 and 12 days. The severity of the DS has been evaluated in the study conducted by PETHEMA, and severe DS usually occurs at the beginning of the treatment (median of 6 days), as compared with moderate DS (median of 15 days). We report here in two cases of late severe DS, with late diagnosis due to both time and form of presentation. We discuss the physiopathology, clinical presentation, prophylaxis and treatment of DS.
Autores principais:Cabral, R.
Outros Autores:Caballero, J.; Alonso, S.; Dávila, J.; Cabrero, M.; Caballero, D.; Vázquez, L.; Sánchez-Guijo, F.; López, L.; Cañizo, M.; Mateos, M.; González, M.
Assunto:differentiation syndrome acute promyelocytic leukemia all trans retinoic acid
Ano:2014
País:Portugal
Tipo de documento:artigo
Tipo de acesso:acesso aberto
Instituição associada:Centro Hospitalar Universitário de Santo António
Idioma:inglês
Origem:Repositório Científico da Unidade Local de Saúde de Santo António (ULSSA)
Descrição
Resumo:Detailed knowledge about differentiation syndrome (DS) has remained limited. There are 2 large studies conducted by the Spanish workgroup PETHEMA (Programa Español de Tratamientos en Hematología; Spanish Program on Hematology Treatments) and the European group trial (LPA 96-99 and APL 93) in which the incidence, characteristics, prognostic factors and outcome of patients developing DS are evaluated. Both have described the median time of DS development between 10 and 12 days. The severity of the DS has been evaluated in the study conducted by PETHEMA, and severe DS usually occurs at the beginning of the treatment (median of 6 days), as compared with moderate DS (median of 15 days). We report here in two cases of late severe DS, with late diagnosis due to both time and form of presentation. We discuss the physiopathology, clinical presentation, prophylaxis and treatment of DS.