CD3-positive plasmablastic B-cell neoplasms: a diagnostic pitfall

Mod Pathol. 2018 May;31(5):718-731. doi: 10.1038/modpathol.2017.177. Epub 2018 Jan 12.

Abstract

Rare B-cell neoplasms with plasmablastic differentiation may aberrantly express CD3 by immunohistochemical staining, which places a great challenge for diagnosis. We here studied 17 cases of CD3+ plasmablastic B-cell neoplasms, including 12 plasmablastic lymphomas and 5 plasmablastic plasma cell myelomas. All 17 cases occurred in the extranodal sites with a male predominance (13/17). Four cases were initially misinterpreted by outside institutions, among which three were diagnosed as 'peripheral T-cell lymphoma, not otherwise specified' and one was classified as 'poorly differentiated neuroendocrine carcinoma'. The plasmablastic cells were present in all 17 cases diffusely or in a subset of tumor cells. CD3 expression was mostly diffuse (12/17) and moderate to strong (11/16) with a cytoplasmic staining pattern (14/16). Other T-cell markers were nearly absent, including CD2 (0/10), CD4 (1/13), CD5 (0/14), CD7 (0/11), and CD8 (0/13). CD138 was positive in all 17 cases and CD79a was variably positive in 8 of 14 cases. Only one case had immunoreactivity to CD20 (1/17) and PAX5 (1/12). CD56 expression and EBV infection were detected in 8/15 and 6/17, respectively. No HHV8 infection was noted in all 11 cases tested. Most cases (11/13) revealed either kappa or lambda light chain restriction. Of the nine cases studied, six had clonal IGH rearrangements but no clonal TRG rearrangements. Our study further emphasizes that the accurate classification of CD3+ plasmablastic neoplasms requires thorough morphologic examination, incorporation of more B-cell and T-cell markers in addition to CD3 and CD20, frequent addition of CD138 staining, and utilization of necessary molecular and genetic studies.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • B-Lymphocytes / pathology
  • Biomarkers, Tumor
  • CD3 Complex*
  • Diagnosis, Differential
  • Female
  • Humans
  • Lymphoma, B-Cell / diagnosis*
  • Lymphoma, B-Cell / pathology
  • Male
  • Middle Aged
  • Multiple Myeloma / diagnosis*
  • Multiple Myeloma / pathology
  • Plasmablastic Lymphoma / diagnosis*
  • Plasmablastic Lymphoma / pathology
  • Sex Factors
  • Young Adult

Substances

  • Biomarkers, Tumor
  • CD3 Complex