Molecular histogenesis of posttransplantation lymphoproliferative disorders

Blood. 2003 Nov 15;102(10):3775-85. doi: 10.1182/blood-2003-05-1683. Epub 2003 Aug 7.

Abstract

Posttransplantation lymphoproliferative disorders (PTLDs) represent a serious complication of solid organ transplantation. This study assessed the molecular histogenesis of 52 B-cell monoclonal PTLDs, including 12 polymorphic PTLDs (P-PTLDs), 36 diffuse large B-cell lymphomas (DLBCLs), and 4 Burkitt/Burkitt-like lymphomas (BL/BLLs). Somatic hypermutation (SHM) of immunoglobulin variable (IgV) genes documented that most monoclonal B-cell PTLDs (75% P-PTLDs, 91.3% DLBCLs, 100% BL/BLLs) derive from germinal center (GC)-experienced B cells. B-cell lymphoma 6 (BCL6) mutations occurred in 25% P-PTLDs, 60.6% DLBCLs, and 75.0% BL/BLLs. A first histogenetic category of PTLDs (31.2% DLBCLs) express the BCL6+/multiple myeloma oncogene-1 protein (MUM1-/+)/CD138- profile and mimic B cells experiencing the GC reaction, as also suggested by ongoing SHM in a fraction of these cases. A second subset of PTLDs (66.7% P-PTLDs and 31.2% DLBCLs) display the BCL6-/MUM1+/CD138- phenotype and mimic B cells that have concluded the GC reaction. A third histogenetic category of PTLDs (25.0% P-PTLDs and 31.2% DLBCLs) shows the BCL6-/MUM1+/CD138+ profile, consistent with preterminally differentiated post-GC B cells. Crippling mutations of IgV heavy chain (IgVH) and/or IgV light chain (IgVL) genes, leading to sterile rearrangements and normally preventing cell survival, occur in 4 DLBCLs and 1 BL/BLL that may have been rescued from apoptosis through expression of Epstein-Barr virus (EBV)-encoded latent membrane protein 1 (LMP1). Overall, the histogenetic diversity of monoclonal B-cell PTLDs may help define biologically homogeneous categories of the disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • B-Lymphocytes / pathology
  • Base Sequence
  • Child
  • Child, Preschool
  • Female
  • Gene Rearrangement
  • Genes, Immunoglobulin
  • Herpesvirus 4, Human
  • Humans
  • Immunophenotyping
  • Infant
  • Lymphoproliferative Disorders / etiology*
  • Lymphoproliferative Disorders / pathology
  • Male
  • Middle Aged
  • Molecular Sequence Data
  • Organ Transplantation / adverse effects*
  • Retrospective Studies
  • Somatic Hypermutation, Immunoglobulin
  • Viral Matrix Proteins / physiology

Substances

  • EBV-associated membrane antigen, Epstein-Barr virus
  • Viral Matrix Proteins