https://scholars.lib.ntu.edu.tw/handle/123456789/627951
標題: | Targeted Next-generation Sequencing Reveals a Wide Morphologic and Immunophenotypic Spectrum of Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma | 作者: | Hang, Jen-Fan CHANG-TSU YUAN Chang, Kung-Chao Wang, Ren-Ching Chen, Bo-Jung Hsieh, Pin-Pen Huang, Wan-Ting Chuang, Wen-Yu Chen, Tsung-Wei Yeh, Yi-Chen Lin, Shih-Yao Hsiao, Cheng-Hsiang Chou, Shih-Cheng Tseng, Chih-En Pan, Shien-Tung Chang, Shih-Lung Chuang, Shih-Sung |
關鍵字: | primary intestinal T-cell lymphoma; celiac disease-related enteropathy-associated T-cell lymphoma; monomorphic epitheliotropic intestinal T-cell lymphoma; primary intestinal peripheral T-cell lymphoma; not otherwise specified; SETD2; NATURAL-KILLER-CELL; MALIGNANT HISTIOCYTOSIS; CELIAC-DISEASE; NK-CELL; ENTEROPATHY; FEATURES | 公開日期: | 1-九月-2022 | 出版社: | LIPPINCOTT WILLIAMS & WILKINS | 卷: | 46 | 期: | 9 | 起(迄)頁: | 1207 | 來源出版物: | The American journal of surgical pathology | 摘要: | Primary intestinal T-cell lymphoma (PITL) is highly aggressive and includes celiac disease-related enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), and primary intestinal peripheral T-cell lymphoma, not otherwise specified (ITCL-NOS). MEITL is the most common PITL in Asia, comprising of monomorphic medium-sized cells typically expressing CD8, CD56, and cytotoxic granules. Occasional cases with intermediate features between MEITL and ITCL-NOS are difficult to be classified and warrant further investigation. We collected 54 surgically resected PITLs from Taiwan, with 80% presenting with bowel perforation. The overall outcome was poor with a median survival of 7 months. Based on histopathology (monomorphic vs. pleomorphic) and immunophenotype, we classified these cases into 4 groups: MEITL with typical immunophenotype (n=34), MEITL with atypical immunophenotype (n=5), pleomorphic PITL with MEITL-like immunophenotype (n=6), and ITCL-NOS (n=9). There was no EATL in our cohort. Targeted next-generation sequencing of the first 3 groups showed highly prevalent loss-of-function mutations for SETD2 (85%, 80%, and 83%, respectively) and frequent activating mutations for STAT5B (64%, 60%, and 50%, respectively) and JAK3 (38%, 20%, and 50%, respectively). In contrast, ITCL-NOS cases had less frequent mutations of SETD2 (56%) and STAT5B (11%) and rare JAK3 mutations (11%). Our results suggest that there is a wider morphologic and immunophenotypic spectrum of MEITL as currently defined in the 2017 WHO classification. MEITL with atypical immunophenotype and PITL with MEITL-like immunophenotype shared clinicopathologic and molecular features similar to MEITL but distinct from ITCL-NOS, indicating that such cases may be considered as immunophenotypic or histopathologic variants of MEITL. |
URI: | https://scholars.lib.ntu.edu.tw/handle/123456789/627951 | ISSN: | 0147-5185 | DOI: | 10.1097/PAS.0000000000001914 |
顯示於: | 醫學院附設癌醫中心醫院(臺大癌醫) |
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