Surface CD3-negative monomorphic epitheliotropic intestinal T-cell lymphoma

  • Domoto Hideharu
    Department of Diagnostic Pathology, Keiyu Hospital, Yokohama, Kanagawa, Japan,
  • Araki Takahiro
    Department of Hematology, Keiyu Hospital, Yokohama, Kanagawa, Japan,
  • Ogai Asuka
    Department of Hematology, Keiyu Hospital, Yokohama, Kanagawa, Japan,
  • Inukai Michiko
    Department of Surgery, Keiyu Hospital, Yokohama, Kanagawa, Japan,
  • Chen Chien K
    Department of Hematology, Keiyu Hospital, Yokohama, Kanagawa, Japan,
  • Tomita Sakura
    Department of Pathological Diagnostics, Tokai University School of Medicine, Isehara, Kanagawa, Japan
  • Mukai Kiyoshi
    Department of Diagnostic Pathology, Keiyu Hospital, Yokohama, Kanagawa, Japan,
  • Nakamura Naoya
    Department of Pathological Diagnostics, Tokai University School of Medicine, Isehara, Kanagawa, Japan

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説明

<p>Intestinal T/NK-cell lymphomas include enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), indolent T-cell lymphoproliferative disorders of the GI tract (ITCLPD), extranodal NK/T-cell lymphoma, nasal type (ENKTL), and intestinal T-cell lymphoma NOS (ITCL-NOS). Here we describe a case of surface CD3-negative MEITL. A 63-year-old Japanese female had a tumor located in the conglomerated ileum, which formed multiple mass lesions. The resected tissue showed a diffuse infiltration of monomorphic medium-sized lymphocytes with epitheliotropism. Flowcytometry using a fresh specimen of the tumor revealed positivity for CD7, CD8, CD38, and CD56, but not surface CD3. On immunohistochemistry, the tumor showed positivity for cytoplasmic CD3, CD8, CD56, TIA-1, Granzyme B, and perforin. EBER with in situ hybridization was negative. Moreover, H3K36me3, which is negative in MEITL with SETD2-mutation, was positive. This is an important case of MEITL due to its oncogenesis.</p>

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