Post-transplant EBV-negative anaplastic large-cell lymphoma with dual rearrangement: a propos of two cases and review of the literature

J Cutan Pathol. 2007 Dec:34 Suppl 1:1-8. doi: 10.1111/j.1600-0560.2006.00712.x.

Abstract

Background: Post-transplant lymphoproliferative disease (PTLD) is a recognized complication of the immunosuppressive regimens associated with solid organ transplantation. The vast majority of these lesions represent monomorphic B-cell lymphoproliferative disease. Rarely, however, T-cell malignancies may emerge, the commonest being anaplastic large-cell lymphoma (ALCL).

Materials and methods: We describe two patients who developed a post-transplant ALCL several years after transplantation. Comprehensive phenotypic and molecular studies were conducted. The technique of capillary gel electrophoresis was employed.

Results: One patient died of unrelated causes, while the other patient did achieve clinical remission. The neoplastic cell populace was composed of CD4-positive cytotoxic T cells exhibiting CD30 positivity. There were very few B cells. Striking and prominent clonally restricted infiltrates were identified whereby there was both a heavy chain and T-cell beta gene rearrangement. There was no evidence of lytic Epstein-Barr virus (EBV) infection.

Conclusion: T-cell-associated PTLD does not appear to be directly attributable to EBV infection. Iatrogenic immune dysregulation may result in excessive T-cell proliferation to various antigenic stimuli, hence resembling other drug-associated cell lymphoproliferative conditions such as angioimmunoblastic lymphadenopathy. The dual rearrangement may have some implications regarding the cell of origin.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antigens, CD / metabolism
  • DNA, Neoplasm / analysis
  • Electrophoresis, Capillary
  • Fatal Outcome
  • Female
  • Gene Rearrangement, B-Lymphocyte, Heavy Chain / genetics*
  • Gene Rearrangement, beta-Chain T-Cell Antigen Receptor / genetics*
  • Herpesvirus 4, Human / isolation & purification*
  • Humans
  • Immunocompromised Host
  • Immunoglobulin Heavy Chains / genetics
  • Liver Transplantation / adverse effects*
  • Lymphoma, Large-Cell, Anaplastic / genetics
  • Lymphoma, Large-Cell, Anaplastic / pathology*
  • Lymphoma, Large-Cell, Anaplastic / virology
  • Male
  • Middle Aged
  • Postoperative Complications*
  • Remission Induction
  • Skin Neoplasms / genetics
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / virology

Substances

  • Antigens, CD
  • DNA, Neoplasm
  • Immunoglobulin Heavy Chains