title: Long-Term Morbidity and Mortality in Children with Chronic Graft-versus-Host Disease Classified by National Institutes of Health Consensus Criteria after Allogeneic Hematopoietic Stem Cell Transplantation.
abstract: We report the long-term morbidity and mortality of 105 pediatric patients who developed chronic graft-versus-host disease (cGVHD) after allogeneic hematopoietic stem cell transplantation (HSCT). According to the consensus criteria of the National Institutes of Health, the global severity of cGVHD was mild in 26 patients (25%), moderate in 30 patients (29%), and severe in 49 patients (47%). Patients with severe cGVHD had a significantly lower cumulative incidence of cGVHD remission and higher probability of continuing cGVHD at 8 years from cGVHD diagnosis compared with those with mild or moderate cGVHD. The 10-year cumulative incidence of nonrelapse mortality in severe cGVHD patients was significantly higher and the probability of disease-free survival was significantly lower than those among patients with mild and moderate cGVHD. Of the 59 patients who survived for more than 5 years, 20 (34%) (4 with moderate and 16 with severe cGVHD) had persistent functional impairment caused by cGVHD with a Karnofsky/Lansky performance score of 90% in 3 patients, 80% in 4 patients, and below 70% in 13 patients at the time of relapse, death, or last follow-up. Better therapeutic strategies are needed to lower the incidence of severe cGVHD, considering the longer life expectancy of pediatric HSCT survivors.
authors.affiliation: Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan. Electronic address: inagakij@nk-cc.go.jp.
authors.forename: Jiro
authors.initials: J
authors.lastname: Inagaki
authors.affiliation: Division of Pediatrics, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Moritake
authors.affiliation: Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental of Sciences, Kagoshima, Japan.
authors.forename: Takuro
authors.initials: T
authors.lastname: Nishikawa
authors.affiliation: Division of Pediatrics, Center of Bone Marrow Transplantation, Hospital of University of the Ryukyus, Okinawa, Japan.
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Hyakuna
authors.affiliation: Department of Pediatrics, Nagasaki University School of Medicine, Nagasaki, Japan.
authors.forename: Masahiko
authors.initials: M
authors.lastname: Okada
authors.affiliation: Division of General Pediatrics and Emergency Medicine, Department of Pediatrics, Oita University Faculty of Medicine, Oita, Japan.
authors.forename: So-ichi
authors.initials: S
authors.lastname: Suenobu
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Saga University, Saga, Japan.
authors.forename: Kozo
authors.initials: K
authors.lastname: Nagai
authors.affiliation: Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Honda
authors.affiliation: Department of Pediatrics, Yamaguchi University Hospital, Yamaguchi, Japan.
authors.forename: Maiko
authors.initials: M
authors.lastname: Shimomura
authors.affiliation: Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Reiji
authors.initials: R
authors.lastname: Fukano
authors.affiliation: Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Maiko
authors.initials: M
authors.lastname: Noguchi
authors.affiliation: Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Koichiro
authors.initials: K
authors.lastname: Kurauchi
authors.affiliation: Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Tanioka
authors.affiliation: Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Okamura
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D032921:Consensus; D005260:Female; D006086:Graft vs Host Disease / Q000276:immunology / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D019337:Hematologic Neoplasms / Q000276:immunology / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D018380:Hematopoietic Stem Cell Transplantation; D006650:Histocompatibility Testing; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D019653:Myeloablative Agonists / Q000627:therapeutic use*; D009316:National Institutes of Health (U.S.); D012189:Retrospective Studies; D012720:Severity of Illness Index; D016019:Survival Analysis; D009626:Terminology as Topic; D014019:Tissue Donors; D019172:Transplantation Conditioning / Q000379:methods*; D014184:Transplantation, Homologous; D014481:United States
chemical_list: D007166:Immunosuppressive Agents; D019653:Myeloablative Agonists
keywords: Children; Chronic graft-versus-host disease; Functional impairment; National Institutes of Health consensus criteria; Nonrelapse mortality; Performance status