Korean J Gastroenterol.  2021 Jul;78(1):53-58. 10.4166/kjg.2021.046.

Two Lethal Cases of Monomorphic Epitheliotropic Intestinal T-cell Lymphoma Deteriorated Rapidly After Emergency Surgery for Intestinal Perforation

• Ko S
• Park SH

• KMID: 2518754
• DOI: http://doi.org/10.4166/kjg.2021.046

Abstract

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive form of primary gastrointestinal T-cell lymphoma. Symptoms can vary but often include fever, abdominal pain, weight loss, diarrhea, obstruction, and perforation. Disease-specific symptoms rarely present before patients reach an advanced stage, which contributes to delayed diagnosis and poor survival outcomes. Approximately half of the patients with MEITL undergo emergency surgery for acute intestinal obstruction or perforation, leading to peritonitis, septic shock, and multiple organ failure. These factors contribute to treatment delays, which are associated with a worse prognosis, particularly in the case of chemotherapy. This paper reports two fatal cases of patients with MEITL who deteriorated rapidly after emergency surgery for intestinal perforation. Patient 1 complained of persistent diarrhea, but a delayed diagnosis led to bowel perforation, and the subsequent chemotherapy treatment was canceled. Patient 2 was diagnosed relatively early, but treatment was delayed due to intestinal perforation. Despite its rarity, MEITL should be considered through a “high index of suspicion” approach when a patient complains of unexplained abdominal pain and diarrhea. This is expected to improve early diagnosis and ultimately patient prognosis.

Keyword

Monomorphic epitheliotropic intestinal T-cell lymphoma; Lymphoma; T-cell; peripheral; Diarrhea; Intestinal perforation

Figure

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  Fig. 1 Colonoscopy showed a focal irregular mucosal thickening in the sigmoid colon.

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  Fig. 2 Abdominal computed tomography demonstrated segmental thickening of the small intestine and sigmoid colon walls.

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  Fig. 3 (A) Microscopically, there was intraepithelial infiltration by small-sized atypical lymphocytes in peripheral zone (×200, H&E). Immunohistochemistry reveals cell wall positivity for the following markers: (B) CD3 (×200), (D) CD8 (×200), (E) CD56 (×200), (F) Ki-67 (×200) while CD20 (not presented), (C) CD4 are negative (×200).

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  Fig. 4 Abdominal computed tomography showed segmental wall thickening of the small intestine and lymph node thickening in the peritoneum.

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  Fig. 5 Colonoscopy showed an ulceroinfiltrative mucosal lesion surrounding half of the cavity in the ileum.

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  Fig. 6 (A) Microscopically, the biopsy showed infiltration of crypt epithelium by small-sized monomorphic lymphocytes (×400, H&E). Immunohistochemically, the cells were (B) CD3+ (×200), (C) CD20-(×200), CD4-(not presented) (D) CD8+ (×200), (E) CD56+ (×200) and (F) Ki-67 index was over 90% (×200).

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