title: Long-term outcome of childhood aplastic anemia patients who underwent allogeneic hematopoietic SCT from an HLA-matched sibling donor in Japan.
abstract: We report long-term outcomes of 329 childhood severe aplastic anemia (SAA) patients who underwent hematopoietic SCT (HSCT) from an HLA-matched sibling donor in the Japanese Hematopoietic Cell Transplantation Registry. OS and EFS at 10 years were as high as 89.7+/-1.7% and 85.5+/-2.0%, respectively. Five cases of late malignancies (LM) were identified (malignant peripheral nerve sheath tumor, thyroid carcinoma, colon carcinoma, MDS and hepatoblastoma). Cumulative incidence of LM was 0.8% at 10 years and 2.5% at 20 years, respectively, which was lower than that in previous reports. This low incidence is in keeping with the low occurrence of skin cancer in Japanese population and of acute GVHD in our study group. Radiation-containing conditioning was not significantly associated with the incidence of LM after HSCT probably because of absolute low patient number who developed LM in our series. In terms of LM development after HSCT, low-dose TBI in HSCT for SAA to avoid graft rejection, which is commonly used in Japan, might be tolerable in the Japanese population because of its low incidence.
authors.affiliation: Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan. akikuchi@med.teikyo-u.ac.jp
authors.forename: A
authors.initials: A
authors.lastname: Kikuchi
authors.forename: H
authors.initials: H
authors.lastname: Yabe
authors.forename: K
authors.initials: K
authors.lastname: Kato
authors.forename: K
authors.initials: K
authors.lastname: Koh
authors.forename: J
authors.initials: J
authors.lastname: Inagaki
authors.forename: Y
authors.initials: Y
authors.lastname: Sasahara
authors.forename: R
authors.initials: R
authors.lastname: Suzuki
authors.forename: N
authors.initials: N
authors.lastname: Yoshida
authors.forename: K
authors.initials: K
authors.lastname: Kudo
authors.forename: R
authors.initials: R
authors.lastname: Kobayashi
authors.forename: K
authors.initials: K
authors.lastname: Tabuchi
authors.forename: K
authors.initials: K
authors.lastname: Kawa
authors.forename: S
authors.initials: S
authors.lastname: Kojima
mesh_terms: D000293:Adolescent; D000741:Anemia, Aplastic / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006680:HLA Antigens / Q000276:immunology; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D007223:Infant; D007564:Japan; D008297:Male; D035781:Siblings; D016019:Survival Analysis; D014019:Tissue Donors; D016896:Treatment Outcome
chemical_list: D006680:HLA Antigens