title: Late differentiation syndrome in acute promyelocytic leukemia: a challenging diagnosis.
abstract: 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.
authors.affiliation: Hematology Service, Hospital Santo António , Porto, Portugal.
authors.forename: Renata
authors.initials: R
authors.lastname: Cabral
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: Caballero
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Sara
authors.initials: S
authors.lastname: Alonso
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Julio
authors.initials: J
authors.lastname: Dávila
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Monica
authors.initials: M
authors.lastname: Cabrero
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Dolores
authors.initials: D
authors.lastname: Caballero
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Lourdes
authors.initials: L
authors.lastname: Vázquez
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Fermin
authors.initials: F
authors.lastname: Sánchez-Guijo
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Lucia
authors.initials: L
authors.lastname: López
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Maria C
authors.initials: MC
authors.lastname: Cañizo
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Maria V
authors.initials: MV
authors.lastname: Mateos
authors.affiliation: Hematology Service, Hospital Universitário , Salamanca, Spain.
authors.forename: Marcos
authors.initials: M
authors.lastname: González
keywords: acute promyelocytic leukemia; all trans retinoic acid; differentiation syndrome