Acute graft-versus-host disease in patients with Fanconi anemia or acquired aplastic anemia undergoing bone marrow transplantation from HLA-identical sibling donors: risk factors and influence on outcome
| Autor: | Agnès Devergie, Patricia Ribaud, Patrice Richard, Anne Janin, R. Traineau, Philippe Guardiola, Xiaxin Li, Eliane Gluckman, Gérard Socié, Helene Esperou |
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| Rok vydání: | 2003 |
| Předmět: |
Adult
Male medicine.medical_specialty Cyclophosphamide Adolescent Anemia Immunology Graft vs Host Disease Biochemistry Gastroenterology Disease-Free Survival Fanconi anemia HLA Antigens Risk Factors hemic and lymphatic diseases Internal medicine Neoplasms medicine Humans Risk factor Child Bone Marrow Transplantation Retrospective Studies business.industry Incidence (epidemiology) Anemia Aplastic Retrospective cohort study Cell Biology Hematology medicine.disease Surgery Transplantation surgical procedures operative Fanconi Anemia Treatment Outcome Relative risk Acute Disease Female business medicine.drug |
| Zdroj: | Blood. 103(1) |
| ISSN: | 0006-4971 |
| Popis: | To assess whether Fanconi anemia (FA) patients might be at risk for acute graft-versus-host disease (AGvHD) despite using low-intensity conditionings, we retrospectively analyzed the incidence of AGvHD and its impact on outcome in 37 FA patients and 73 patients with acquired aplastic anemia (AAA) that received transplants at Saint Louis Hospital from HLA-genotypic identical siblings with similar conditionings (thoraco-abdominal irradiation plus cyclophosphamide 20 [FA] or 150 mg/kg [AAA]). Despite being younger, FA patients had an increased risk of grades II to IV AGvHD (relative risk [RR], 2.00; P = .021), especially in younger patients (RR, 7.93; P = .014). The risks of requiring systemic corticosteroids to treat AGvHD and experiencing cortico-resistant AGvHD were significantly increased in FA patients. Although non-FA and FA patients had similar 10-year outcomes, acute and chronic GvHD had a biphasic effect on FA patient outcome with an additional cluster of lethal events starting by 5 years after transplantation. This late survival fall, restricted to FA patients, was closely related to head and neck carcinomas (15-year incidence: 53%). FA patients represent a group at risk regarding AGvHD when using irradiation-based conditionings. The impact of AGvHD on survival may not be limited to the early posttransplantation period and may be a major risk factor for head and neck carcinomas and late mortality in FA patients. |
| Databáze: | OpenAIRE |
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