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<p>Primary effusion lymphoma (PEL) is a rare and aggressive non-Hodgkin lymphoma that primarily involves body cavities and is characterized by human herpes virus-8 (HHV-8) positivity and the presence of immunoblastic, plasmablastic or anaplastic cells. PEL typically lacks B-cell antigens, but involves immunoglobulin gene rearrangements, suggesting a B-cell origin. Here, we present a highly unusual case of PEL in which tumor cells expressed T-cell antigens but were also monoclonal for both T-cell receptor (TCR) and immunoglobulin heavy locus (IGH) gene rearrangements. An 81-year-old man with bilateral pleural effusions presented with atypical lymphoid cells. Immunohistochemistry revealed positivity for CD3, CD4, MUM-1, OCT2, BOB1, and HHV-8, but negativity for other T- and B-cell antigens. In situ hybridization identified Epstein-Barr virus and lambda light chain restriction. Gene rearrangement studies demonstrated monoclonal <i>TRB</i> (T-cell receptor beta locus) and <i>TRG</i> (T-cell receptor gamma locus) as well as <i>IGH</i> rearrangement. This unusual case of PEL with T-cell antigen expression and clonal rearrangement of both the <i>TCR</i> and <i>IGH</i> genes highlights the lineage complexity of this tumor.</p>

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Primary effusion lymphoma with biphenotypic and bigenotypic features of T-cell receptor and IGH genes in an HIV-negative patient with kidney transplant

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年9月19日

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  • Taesung Jeon,
  • Young-Hyeh Ko,
  • You-Na Sung,
  • Hyunsung Kim,
  • Ka-Won Kang,
  • Jong-Min Sim,
  • Yeseul Kim

摘要

Primary effusion lymphoma (PEL) is a rare and aggressive non-Hodgkin lymphoma that primarily involves body cavities and is characterized by human herpes virus-8 (HHV-8) positivity and the presence of immunoblastic, plasmablastic or anaplastic cells. PEL typically lacks B-cell antigens, but involves immunoglobulin gene rearrangements, suggesting a B-cell origin. Here, we present a highly unusual case of PEL in which tumor cells expressed T-cell antigens but were also monoclonal for both T-cell receptor (TCR) and immunoglobulin heavy locus (IGH) gene rearrangements. An 81-year-old man with bilateral pleural effusions presented with atypical lymphoid cells. Immunohistochemistry revealed positivity for CD3, CD4, MUM-1, OCT2, BOB1, and HHV-8, but negativity for other T- and B-cell antigens. In situ hybridization identified Epstein-Barr virus and lambda light chain restriction. Gene rearrangement studies demonstrated monoclonal TRB (T-cell receptor beta locus) and TRG (T-cell receptor gamma locus) as well as IGH rearrangement. This unusual case of PEL with T-cell antigen expression and clonal rearrangement of both the TCR and IGH genes highlights the lineage complexity of this tumor.