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Lymphoproliferative disorders after organ transplantation in children
Y Dror1, M Greenberg, G Taylor
1Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Pediatric posttransplant lymphoproliferative disorders (PTLD) can be treated effectively with immunosuppressive drug reduction and antiviral therapy. The Society for Hematopathology Workshop (SHPW) classification shows clinical relevance but requires modification for accurate clonality assessment.
Area of Science:
- Pediatric Hematology
- Transplant Immunology
- Oncology
Background:
- Posttransplant lymphoproliferative disorders (PTLD) are a significant risk for organ transplant recipients.
- This study focuses on pediatric PTLD cases, analyzing incidence, clinical course, and outcomes.
Purpose of the Study:
- To analyze 26 pediatric PTLD cases diagnosed between 1988 and 1996.
- To evaluate the applicability of the 1997 Society for Hematopathology Workshop (SHPW) classification in pediatric PTLD.
Main Methods:
- Retrospective chart review for incidence, clinical course, and outcomes.
- Pathological classification of tissue samples using SHPW criteria.
- Morphological, molecular, and immunophenotypic assessments of PTLD lineage.
Main Results:
- PTLDs were classified morphologically as monomorphic (20), polymorphic (5), and hyperplastic (1).
- Immunophenotyping revealed B cell (12), T cell (4), mixed B/T cell (8), and undetermined (2) lineages.
- Treatment with immunosuppressive drug reduction, antivirals, surgery, and irradiation led to complete remission in 13 patients and partial remission in 3. Adverse prognostic factors included low platelet/neutrophil counts and advanced stage.
Conclusions:
- Most pediatric PTLD patients can achieve cure with reduced immunosuppression, antivirals, and localized treatments.
- The SHPW morphologic grouping is feasible and clinically relevant.
- Modifications are needed to improve correlation between morphology, clonality, and cell origin assessments.
Background:
After organ transplant, patients are at risk of posttransplant lymphoproliferative disorders (PTLD). The purpose of this study was to analyze 26 pediatric cases of PTLD observed at our institution between 1988 and 1996, and to evaluate the validity of the Society for Hematopathology Workshop (SHPW) 1997 classification in our patient population.
Methods:
Charts were reviewed for analysis of incidence, clinical course, and outcome. Tissue samples were classified by a pathologist according to SHPW recommendations.
Results:
By morphology, 20 were monomorphic, 5 polymorphic, and 1 hyperplastic. Assessment of lineage by morphology, molecular studies, and immunophenotyping did not correlate in six cases. By immunophenotyping, 12 were B cell, 4 T cell, 8 mixed B/T cells, and 2 undetermined. The 20 patients evaluable for treatment efficacy were treated with various therapeutic combinations, including immunosuppressive drug reduction, acyclovir/ganciclovir, interferon-alpha, immunoglobulins, surgery, and local irradiation. No patient received systemic chemotherapy. Thirteen patients achieved complete remission and 3, partial; 1 died 5 days after starting therapy, and 3 of progressive disease. Adverse prognostic factors included low platelet or neutrophil counts; stage III-IV and SHPW morphology were marginally significant.
Conclusions:
The majority of patients eligible for treatment can be cured with immunosuppressive drug reduction and antiviral drugs, along with surgery and irradiation when indicated. Systemic chemotherapy or innovative approaches may have a role in unresponsive cases. Morphologic SHPW grouping is feasible and seems to have clinical relevance. However, correlation with clonality and immunophenotyping is not always possible, necessitating modifications including segregation of descriptive morphology from clonality and cell origin.