Department of Hematology, the First Hospital of China Medical University, Shenyang, China.
J Int Med Res. 2024 Aug;52(8):3000605241271756. doi: 10.1177/03000605241271756.
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), also known as type II enteropathy-associated T-cell lymphoma, is a rare malignant lymphoma of the extranodal lymphoid tissue derived from interepithelial T lymphocytes. MEITL is a primary intestinal T-cell lymphoma with a challenging diagnosis and aggressive progression, and it can invade other extraintestinal sites. In this study, we report four patients diagnosed with MEITL. All patients presented with abdominal pain, and one patient was admitted because of acute intestinal perforation. Two patients presented with unformed defecation and diarrhea. All patients carried the immunophenotypes CD3, CD7, CD8, CD20, and CD56, and the Ki-67 index ranged 60% to 90%. Three cases were analyzed using next-generation sequencing. One case displayed possibly relevant alterations of CREBBP, NOTCH2, SETD2, and STAT5B, and another case exhibited definite alteration of NOTCH1, possibly relevant alterations of CCND1 and DNMT3A, and potentially relevant alterations of HISTH3B, IGLL5, KMT2C, and KRAS. Different chemotherapy regimens were used, but the prognosis was poor. Hence, we illustrated that because of its low incidence, challenging diagnosis, and difficult treatment, further therapeutic improvements are urgently warranted.
单一表型上皮样肠道 T 细胞淋巴瘤（MEITL），又称 II 型肠病相关 T 细胞淋巴瘤，是一种罕见的来源于上皮间 T 淋巴细胞的结外淋巴组织恶性淋巴瘤。MEITL 是一种原发性肠道 T 细胞淋巴瘤，具有诊断挑战性和侵袭性进展，可侵犯其他肠道外部位。本研究报道了 4 例 MEITL 患者。所有患者均以腹痛为首发症状，其中 1 例因急性肠穿孔入院。2 例表现为不成形排便和腹泻。所有患者均表达免疫表型 CD3、CD7、CD8、CD20 和 CD56，Ki-67 指数为 60%至 90%。3 例进行了下一代测序分析。1 例显示 CREBBP、NOTCH2、SETD2 和 STAT5B 可能存在相关改变，另 1 例显示 NOTCH1 明确改变，CCND1 和 DNMT3A 可能存在相关改变，HISTH3B、IGLL5、KMT2C 和 KRAS 可能存在相关改变。采用了不同的化疗方案，但预后较差。因此，我们表明由于其发病率低、诊断困难和治疗困难，迫切需要进一步的治疗改善。
