Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Füreder, A; Kropshofer, G; Benesch, M; Dworzak, M; Greil, S; Huber, WD; Hubmann, H; Lawitschka, A; Mann, G; Michel-Behnke, I; Müller-Sacherer, T; Pichler, H; Simonitsch-Klupp, I; Schwinger, W; Szepfalusi, Z; Crazzolara, R; Attarbaschi, A, , Austrian, Society, of, Pediatric, Hematology, and, Oncology.
Characteristics, management, and outcome of pediatric patients with post-transplant lymphoproliferative disease-A 20 years' experience from Austria.
Cancer Rep (Hoboken). 2021; 4(5):e1375
Doi: 10.1002/cnr2.1375
[OPEN ACCESS]
Web of Science
PubMed
FullText
FullText_MUG
- Co-Autor*innen der Med Uni Graz
- Benesch Martin
- Hubmann Holger
- Schwinger Wolfgang
- Altmetrics:
- Dimensions Citations:
- Plum Analytics:
- Scite (citation analytics):
- Abstract:
- BACKGROUND: Management of pediatric post-transplantation lymphoproliferative disorder (PTLD) after hematopoietic stem cell (HSCT) and solid organ transplantation (SOT) is challenging. AIM: This study of 34 PTLD patients up to 19-years old diagnosed in Austria from 2000 to 2018 aimed at assessing initial characteristics, therapy, response, and outcome as well as prognostic markers of this rare pediatric disease. METHODS AND RESULTS: A retrospective data analysis was performed. Types of allografts were kidney (n = 12), liver (n = 7), heart (n = 5), hematopoietic stem cells (n = 4), lungs (n = 2), multi-visceral (n = 2), small intestine (n = 1), and vessels (n = 1). Eighteen/34 were classified as monomorphic PTLD, with DLBCL accounting for 15 cases. Polymorphic disease occurred in nine, and non-destructive lesions in six cases. One patient had a non-classifiable PTLD. Thirteen/34 patients are surviving event-free in first remission (non-destructive, n = 4/6; polymorphic, n = 4/9; monomorphic, n = 6/18). Fourteen/34 patients lacked complete response to first-line therapy, of whom seven died. Four/34 patients relapsed, of whom two died. In 3/34 patients, death occurred as a first event. The 5-year overall and event-free survival rates were 64% ± 9% and 35% ± 9% for the whole cohort. Among all parameters analyzed, only malignant disease as the indication for transplantation had a significantly poor influence on survival. CONCLUSIONS: This study shows PTLD still to be a major cause of mortality following SOT or HSCT in children. A continued understanding of the molecular biology of the disease shall allow to decrease treatment intensity for lower risk patients and to identify patients who may benefit from newer therapy approaches to improve outcome and decrease morbidity.
- Find related publications in this database (using NLM MeSH Indexing)
- Adolescent - administration & dosage
- Adult - administration & dosage
- Austria - epidemiology
- Child - administration & dosage
- Child, Preschool - administration & dosage
- Disease Management - administration & dosage
- Female - administration & dosage
- Follow-Up Studies - administration & dosage
- Hematologic Neoplasms - pathology, therapy
- Hematopoietic Stem Cell Transplantation - adverse effects
- Humans - administration & dosage
- Infant - administration & dosage
- Infant, Newborn - administration & dosage
- Lymphoproliferative Disorders - epidemiology, etiology, mortality, pathology
- Male - administration & dosage
- Organ Transplantation - adverse effects
- Prognosis - administration & dosage
- Retrospective Studies - administration & dosage
- Survival Rate - administration & dosage
- Transplantation, Homologous - administration & dosage
- Young Adult - administration & dosage
- Find related publications in this database (Keywords)
- hematopoietic stem cell transplantation
- outcome
- post‐
- transplant lymphoproliferative disease
- solid organ transplantation
- treatment