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Peripheral T-cell lymphoma in children and adolescents: role of bone marrow transplantation
B G Gordon1, D D Weisenburger, W G Sanger
1Department of Pediatrics, University of Nebraska Medical Center, Omaha 68198-2168.
Insights
Pediatric peripheral T-cell lymphoma (PTCL) differs from adult PTCL, with more frequent CD30 expression. High-dose chemoradiotherapy with stem cell transplant shows promise for treating pediatric PTCL.
Area of Science:
- Pediatric Oncology
- Hematology
- Immunology
Background:
- Peripheral T-cell lymphoma (PTCL) presents diverse features in children and adults.
- Significant differences exist in clinical, morphologic, cytogenetic, and immunologic characteristics between pediatric and adult PTCL.
- CD30 expression is notably more prevalent in pediatric PTCL compared to adult cases.
Purpose of the Study:
- To delineate the distinct features of PTCL in pediatric populations.
- To evaluate treatment efficacy for pediatric PTCL.
- To identify specific markers and prognostic indicators in childhood PTCL.
Main Methods:
- Comparative analysis of clinical, morphologic, cytogenetic, and immunologic data from pediatric PTCL patients.
- Assessment of CD30 expression and t(2;5) translocation frequency.
- Evaluation of treatment outcomes, including conventional chemotherapy and high-dose chemoradiotherapy with hematopoietic stem cell transplantation.
Main Results:
- Pediatric PTCL frequently exhibits CD30 expression, often without anaplastic large cell histology.
- The t(2;5) translocation is not a specific marker for anaplastic large cell lymphoma in children.
- Conventional chemotherapy post-relapse is generally ineffective for pediatric PTCL.
- High-dose chemoradiotherapy followed by hematopoietic stem cell transplantation appears effective, particularly a regimen including thioTEPA, VP-16, and total body irradiation.
Conclusions:
- Pediatric PTCL exhibits unique characteristics, including higher CD30 expression rates.
- Effective treatment strategies for pediatric PTCL may involve high-dose chemoradiotherapy and stem cell transplantation.
- Further research is essential to understand and optimize the management of this rare pediatric malignancy.
Abstract:
Although PTCL in children, as in adults, has a spectrum of clinical, morphologic, cytogenetic and immunologic features, there are several significant differences in these features between children and adults. Our data show that CD30 expression is much more common in pediatric PTCL than is reported in adult PTCL. Furthermore, the majority of children with CD30-positive PTCL do not have tumors with anaplastic large cell histology. Our data also suggest that the t(2;5) is not a specific marker of anaplastic large cell lymphoma in childhood. The likelihood of cure for children with PTCL is unclear, predominantly because of the lack of large numbers of pediatric patients with this less common entity. As with other NHL, we expect that treatment with conventional dose chemotherapy following relapse will be unsuccessful in most cases. Although the data are preliminary, it appears that high dose chemoradiotherapy followed by hematopoietic stem cell transplantation is an effective therapy in these patients. We have been particularly successful with a regimen based on thioTEPA, VP-16 and total body irradiation, but other regimens may also be efficacious. Further studies of this interesting group of tumors are clearly needed.