Assuntos
Infecções por Vírus Epstein-Barr , Herpesvirus Humano 4/imunologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Transtornos Linfoproliferativos , Segunda Neoplasia Primária , Adolescente , Linfócitos B/imunologia , Infecções por Vírus Epstein-Barr/etiologia , Infecções por Vírus Epstein-Barr/imunologia , Infecções por Vírus Epstein-Barr/terapia , Feminino , Humanos , Falência Renal Crônica/imunologia , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/imunologia , Transtornos Linfoproliferativos/terapia , Transtornos Linfoproliferativos/virologia , Segunda Neoplasia Primária/etiologia , Segunda Neoplasia Primária/imunologia , Segunda Neoplasia Primária/terapia , Segunda Neoplasia Primária/virologia , Linfócitos T/imunologiaRESUMO
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.
Assuntos
Doença Enxerto-Hospedeiro/etiologia , Transplante de Células-Tronco Hematopoéticas , Doenças da Boca/etiologia , Adolescente , Glucocorticoides/uso terapêutico , Doença Enxerto-Hospedeiro/tratamento farmacológico , Humanos , Masculino , Doenças da Boca/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células T Precursoras/terapia , Prednisona/uso terapêuticoRESUMO
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.