CD123-positive plasmacytoid dendritic cells in primary cutaneous marginal zone B-cell lymphoma: diagnostic and pathogenetic implications

Am J Surg Pathol. 2009 Sep;33(9):1307-13. doi: 10.1097/pas.0b013e3181a6ae1e.

Abstract

The histogenesis of primary cutaneous marginal zone B-cell lymphoma (PCMZL) has not yet been clarified. Plasma-cytoid dendritic cells (PDC) play a crucial role in the initiation of immune responses and activation of T-cells and B-cells. We analyzed the presence of PDC by immunohistochemistry in various forms of primary cutaneous B-cell lymphomas and in B-cell pseudolymphomas. Clusters of CD123+PDC were observed in all PCMZL (23 of 23 cases; 100%) and in two-thirds of cutaneous B-cell pseudolymphomas (14 of 22; 64%), but only in a minority of primary cutaneous follicle center lymphoma (4 of 31; 13%) and not in primary cutaneous diffuse large B-cell lymphoma, leg type. CD123+ PDC clusters were found in close proximity to monoclonal plasma cells and T-cells and were already present at high numbers in early lesions and initial recurrences of PCMZL. In conclusion, the detection of larger clusters of PDC in PCMZL provides a useful adjunctive diagnostic marker and suggests a pathogenetically relevant role of these cells in PCMZL. Furthermore, the occurrence of PDC could explain the high number of T-cells typically found in PCMZL. We propose considering PCMZL as a unique and potentially antigen-driven neoplasm with PDC, T-cells, and B-cells as the constitutive tumor components.

MeSH terms

  • Biomarkers, Tumor / metabolism
  • Clone Cells
  • Dendritic Cells / metabolism*
  • Dendritic Cells / pathology
  • Gene Rearrangement, B-Lymphocyte, Light Chain
  • Humans
  • Immunoenzyme Techniques
  • Immunoglobulin Light Chains / metabolism
  • Interleukin-3 Receptor alpha Subunit / metabolism*
  • Lymph Nodes / pathology
  • Lymphoma, B-Cell, Marginal Zone / genetics
  • Lymphoma, B-Cell, Marginal Zone / metabolism*
  • Lymphoma, B-Cell, Marginal Zone / pathology
  • Lymphoma, Follicular / genetics
  • Lymphoma, Follicular / metabolism
  • Lymphoma, Follicular / pathology
  • Lymphoma, Large B-Cell, Diffuse / genetics
  • Lymphoma, Large B-Cell, Diffuse / metabolism
  • Lymphoma, Large B-Cell, Diffuse / pathology
  • Plasma Cells / metabolism*
  • Plasma Cells / pathology
  • Polymerase Chain Reaction
  • Skin Neoplasms / metabolism*
  • Skin Neoplasms / pathology

Substances

  • Biomarkers, Tumor
  • IL3RA protein, human
  • Immunoglobulin Light Chains
  • Interleukin-3 Receptor alpha Subunit