Autopsy case report of intravascular large B-cell lymphoma with neoplastic PD-L1 expression

  • Sakakibara Ayako
    Department of Pathology and Laboratory Medicine, Nagoya University Hospital,
  • Inagaki Yuichiro
    Department of Hematology and Oncology, Anjo Kosei Hospital,
  • Imaoka Eiki
    Department of Neurosurgery, Anjo Kosei Hospital,
  • Ishikawa Eri
    Department of Pathology and Laboratory Medicine, Nagoya University Hospital,
  • Shimada Satoko
    Department of Pathology and Laboratory Medicine, Nagoya University Hospital,
  • Shimada Kazuyuki
    Department of Hematology and Oncology, Nagoya University Graduate School of Medicine,
  • Suzuki Yuka
    Department of Pathology and Laboratory Medicine, Nagoya University Hospital,
  • Nakamura Shigeo
    Department of Pathology and Laboratory Medicine, Nagoya University Hospital,
  • Satou Akira
    Department of Surgical Pathology, Aichi Medical University Hospital
  • Kohno Kei
    Department of Pathology and Laboratory Medicine, Nagoya University Hospital,

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<p>Intravascular large B-cell lymphoma (IVLBCL) is a rare and clinically distinctive entity characterized by the almost exclusive growth of large cells within the lumen of blood vessels in particular capillaries. Reports of this peculiar disease, do not commonly address the PD-L1 expression on IVLBCL tumor cells. Here, we describe a 51-year-old Japanese woman who presented with rapidly progressive cognitive decline and higher brain dysfunction. CT scan and MRI revealed multiple ischemic foci in the cerebral hemispheres, ground-glass opacity in the lungs, and splenomegaly. Random skin biopsy for IVLBCL diagnosis yielded negative results. The patient experienced a rapidly deteriorating clinical course with no treatment, and died from the disease after 3 months of hospitalization. Post-mortem examination revealed systemic intravascular plugging of lymphoma cells, without mass lesions in the central nervous system or in visceral organs such as the lungs, liver, pituitary gland, ovaries, and uterus. The tumor cells were positive for CD10, CD20, BCL2, BCL6, and MUM1, but not other lineage-specific markers. Notably, the tumor cells showed strong PD-L1 expression. Our case was diagnosed as IVLBCL with neoplastic PD-L1 expression. These findings suggest that PD-L1 is associated with immune evasion of IVLBCL and may play a role in the pathogenesis and peculiar biological behavior of this unique disease. Additionally, PD-L1 may represent a possible therapeutic target for immune check-point inhibitors.</p>

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