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Posttransplantation plasma cell dyscrasias
1Division of Hematology/Oncology, Henry Vogt Cancer Research Institute, University of Louisville School of Medicine, Kentucky.
Cancer
|October 1, 1994
Summary
Post-transplant lymphoproliferative disorders with plasmacytic differentiation, though rare, can resolve. This case study shows a patient with Epstein-Barr virus-associated plasmacytomas and monoclonal paraprotein that responded to treatment.
Area of Science:
- Oncology
- Immunology
- Transplantation Medicine
Background:
- Post-transplant lymphoproliferative disorders (PTLD) are typically B-cell neoplasms linked to Epstein-Barr virus (EBV) in immunosuppressed organ transplant recipients.
- While some PTLDs show plasmacytoid features, terminal differentiation into plasma cells secreting monoclonal immunoglobulin is uncommon.
- This report details a liver transplant patient who developed EBV-positive abdominal and bladder plasmacytomas with a significant monoclonal immunoglobulin G kappa paraprotein.
Observation:
- The patient presented with multiple plasmacytomas and elevated levels of a specific monoclonal immunoglobulin.
- Epstein-Barr virus (EBV) genomes and RNA transcripts were detected in the plasmacytomas.
- Clonally restricted kappa light chain expression was observed, but EBV latent membrane protein 1 was not detected, and H-ras gene mutations were absent.
Findings:
- Epstein-Barr virus (EBV) genomes and EBER-1 transcripts confirmed EBV presence in the plasmacytomas.
- Immunohistochemistry revealed clonal kappa light chain restriction, indicating a monoclonal plasma cell proliferation.
- No codon-12 mutations in the H-ras gene were identified in the tumor cells.
Implications:
- The successful resolution of plasmacytomas and paraprotein levels following radiation and reduced immunosuppression suggests a favorable prognosis.
- Terminal plasmacytic differentiation in EBV-associated PTLD does not inherently indicate a poor outcome.
- This case highlights the potential for successful management of rare, differentiated PTLDs in transplant patients.