title: Switch from beta-thalassemia major to beta-thalassemia intermedia after secondary graft failure.
abstract: In this article, we report a switch of beta-thalassemia major to intermedia beta-thalassemia after allogeneic bone marrow transplant of a 6-year-old girl from her HLA-matched brother. After stable mixed chimerism, the patient had a secondary graft rejection and returned to total recipient chimerism as assessed by real-time polymerase chain reaction assay. Nonetheless, with a medium hemoglobin rate of 89 g/L, she did not need further transfusions for 60 months after rejection. We conclude that complete loss of donor cells after bone marrow transplant for beta-thalassemia major is compatible with a stable clinical state, probably due to a gamma-globin gene demethylation that enhances gamma-globin chain production and further allows constitution of a fetal hemoglobin rate compatible with free transfusion survival.
authors: Service d'Immuno-Hématologie pédiatrique, Centre National de Greffe de Moelle Osseuse, Tunis, Tunisia. melloulif@yahoo.fr
authors: Fethi
authors: F
authors: Mellouli
authors: Habib
authors: H
authors: Ksouri
authors: Amel
authors: A
authors: Lakhal
authors: Lamia
authors: L
authors: Torjmen
authors: Abderrahman
authors: A
authors: Abdelkefi
authors: Saloua
authors: S
authors: Ladeb
authors: Slama
authors: S
authors: Hmida
authors: Tarek Ben
authors: TB
authors: Othman
authors: Mohamed
authors: M
authors: Béjaoui
mesh_terms: D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002648:Child; D005190:Family; D005260:Female; D005319:Fetal Hemoglobin / Q000378:metabolism; D006084:Graft Rejection / Q000097:blood / Q000209:etiology* / Q000235:genetics / Q000276:immunology; D006680:HLA Antigens / Q000276:immunology; D006441:Hemoglobin A / Q000378:metabolism; D006443:Hemoglobin A2 / Q000378:metabolism; D006650:Histocompatibility Testing; D006801:Humans; D016133:Polymerase Chain Reaction; D013997:Time Factors; D018183:Transplantation Chimera / Q000235:genetics; D019172:Transplantation Conditioning; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D017086:beta-Thalassemia / Q000097:blood / Q000235:genetics / Q000276:immunology / Q000601:surgery*
chemical_list: D006680:HLA Antigens; D006441:Hemoglobin A; D006443:Hemoglobin A2; D005319:Fetal Hemoglobin