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Case Reports
. 2022 Sep 28;62(3):169-174.
doi: 10.3960/jslrt.22005. Epub 2022 Aug 18.

Surface CD3-negative monomorphic epitheliotropic intestinal T-cell lymphoma

Hideharu Domoto  1 , Takahiro Araki  2 , Asuka Ogai  2 , Michiko Inukai  3 , Chien K Chen  2 , Sakura Tomita  4 , Kiyoshi Mukai  1 , Naoya Nakamura  4
Affiliations
Case Reports

Surface CD3-negative monomorphic epitheliotropic intestinal T-cell lymphoma

Hideharu Domoto et al. J Clin Exp Hematop. .

Abstract

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.

Keywords: flowcytometry; intestinal T/NK-cell lymphoma; monomorphic epitheliotropic intestinal T-cell lymphoma; surface CD3.

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Conflict of interest statement

CONFLICT OF INTEREST

The authors have no potential conflicts of interest to declare with respect to the research, authorship, and/or publication of this article.

Figures

Fig. 1
Fig. 1
Computed tomographic scan without contrast enhancement on admission. An ileal mass with wall thickening is identified (arrow) but intestinal obstruction is not seen (A). Peritonitis is suspected due to thickening of the peritoneum and mild increased intensity of the intrapelvic adipose tissue (ellipse) (B).
Fig. 2
Fig. 2
Gross features of the cut surface of the ileal mass. A whitish protruding tumor with surface erosion and scatted focal necrosis is seen. The ileal wall shows diffuse thickening and tumor infiltrated the mesentery.
Fig. 3
Fig. 3
Lymphoma cells diffusely infiltrated the entire ileal wall (A). Focal coagulated necrosis (B) and angioinvasion (C) are seen. Lymphoma cells are monomorphic and show infiltration into the crypt epithelium (D, epitheliotropism).
Fig. 4
Fig. 4
Immunophenotypes of lymphoma cells. Lymphoma cells are positive for CD3 (A), CD8 (B), CD56 (C), TIA-1 (D), Granzyme B (E), perforin (F), and H3K36me3 (G). The MIB1 index is approximately 40% (H).
Fig. 5
Fig. 5
Flowcytometric analysis of CD45 gated lymphoma cells of the ileal wall. The lymphoma cells are positive for cytoplasmic CD3, CD7, CD8, CD38, and CD56 and negative for CD2, surface CD3, CD4, CD5, CD10, CD19, CD20, and CD23.
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