Asunto(s)
Infecciones por Virus de Epstein-Barr , Herpesvirus Humano 4/inmunología , Fallo Renal Crónico/cirugía , Trasplante de Riñón , Trastornos Linfoproliferativos , Neoplasias Primarias Secundarias , Adolescente , Linfocitos B/inmunología , Infecciones por Virus de Epstein-Barr/etiología , Infecciones por Virus de Epstein-Barr/inmunología , Infecciones por Virus de Epstein-Barr/terapia , Femenino , Humanos , Fallo Renal Crónico/inmunología , Trastornos Linfoproliferativos/etiología , Trastornos Linfoproliferativos/inmunología , Trastornos Linfoproliferativos/terapia , Trastornos Linfoproliferativos/virología , Neoplasias Primarias Secundarias/etiología , Neoplasias Primarias Secundarias/inmunología , Neoplasias Primarias Secundarias/terapia , Neoplasias Primarias Secundarias/virología , Linfocitos T/inmunologíaRESUMEN
Oral acute graft-versus-host disease (aGVHD) is a significant sequelae of allogeneic hematopoietic stem cell transplantation (HSCT). Presently, transplant physicians have to diagnose GVHD based on clinical judgment by interpreting available clinical and relevant laboratory findings. As such, characterization of diagnostic and distinctive clinical signs and symptoms of GVHD is essential for diagnosis and grading. The oral features of aGVHD have been reported infrequently and remain ill defined, unlike in oral chronic GVHD. The report describes an atypical and painful presentation of oral aGVHD in a 15-year-old boy, 16 days after haploidentical HSCT, who presented with swollen lips, herpetiform ulcerations, and erythematous fungiform papilla.
Asunto(s)
Enfermedad Injerto contra Huésped/etiología , Trasplante de Células Madre Hematopoyéticas , Enfermedades de la Boca/etiología , Adolescente , Glucocorticoides/uso terapéutico , Enfermedad Injerto contra Huésped/tratamiento farmacológico , Humanos , Masculino , Enfermedades de la Boca/tratamiento farmacológico , Leucemia-Linfoma Linfoblástico de Células T Precursoras/terapia , Prednisona/uso terapéuticoRESUMEN
We retrospectively analyzed the outcomes of hematopoietic stem cell transplantation in 7 patients with primary immunodeficiency diseases treated at the National University Hospital, Singapore, over the period from December 1996 to January 2010. The primary immunodeficiency diseases managed were X-linked hyperimmunoglobulin M syndrome (n = 3), severe combined immunodeficiency (n = 1), leukocyte adhesion deficiency type 1 (n = 1), chronic granulomatous disease (n = 1), and Wiskott-Aldrich syndrome (n = 1). The age of the patients ranged from 5 months to 17 years. Conditioning regimen depended on the type of immunodeficiency, whereas supportive treatment was tailored for differing pretransplant conditions. Eight stem cell transplantations were performed for 7 patients. Donors were HLA-matched sibling donors for 2 patients and unrelated donors for the rest. At the median follow-up of 8.6 years (range 2.2-15.0 years) as of December 2011, 6 patients were alive and cured of their primary diseases.