原发黏膜CD30阳性T细胞淋巴组织增殖性疾病临床病理学分析  

Clinicopathological features of primary mucosal CD30-positive T-cell lymphoproliferative disorders

作　　者：董娜 张丽娜 郑媛媛[1] 张燕林[1] 周小鸽[1] 谢建兰[1] Dong Na;Zhang Lina;Zheng Yuanyuan;Zhang Yanlin;Zhou Xiaoge;Xie Jianlan(Department of Pathology,Beijing Friendship Hospital Affiliated to Capital Medical University,Beijing 100050,China;Department of Pathology,Beijing Lu Daopei Hospital,Beijing 100176,China)

机构地区：[1]首都医科大学附属北京友谊医院病理科,北京100050 [2]北京陆道培医院病理室,北京100176

出　　处：《中华病理学杂志》2024年第7期667-671,共5页Chinese Journal of Pathology

摘　　要：目的探讨原发黏膜CD30阳性T细胞淋巴组织增殖性疾病(pmCD30+TLPD)的临床病理特征及鉴别诊断。方法回顾性收集首都医科大学附属北京友谊医院及北京陆道培医院2013至2023年会诊的8例pmCD30+TLPD,检测该病的免疫表型、EB病毒感染状态和T细胞受体(TCR)克隆性,分析患者的临床病理特征并复习相关文献。结果患者男性3例,女性5例,年龄28~73岁;均无B症状,缺乏创伤和自身免疫性疾病。7例发生于口腔黏膜,1例为肛管黏膜。所有病例表现为黏膜结节,除1例外均伴溃疡。形态学上,于炎性背景中分布多少不等的异型淋巴细胞,4例呈“肾形”“胚胎样”及“马蹄铁样”细胞;1例类似霍奇金淋巴瘤HRS细胞。异型淋巴细胞表达CD3(7/8)、CD4+/CD8-(7/8)、CD4-/CD8-(1/8);CD30均匀强阳性,不表达间变性淋巴瘤激酶(ALK)和CD56。5/5例EB病毒原位杂交阴性。2例TCR基因重排阳性。4例未进行放化疗。除1例因同时伴有白细胞减少症而死亡外,7例均无病生存。结论pmCD30+TLPD形态学谱系较宽,容易与原发皮肤CD30阳性T细胞淋巴组织增殖性疾病和累及黏膜的系统性ALK阴性间变性大细胞淋巴瘤相混淆,从而造成误诊。预后良好,少数病例复发或进展为淋巴瘤。Objective To investigate the clinicopathological features and differential diagnosis of primary mucosal CD30-positive T-cell lymphoproliferative disorders(pmCD30+TLPD).Methods Eight cases of pmCD30+TLPD diagnosed from 2013 to 2023 at the Department of Pathology,Beijing Friendship Hospital Affiliated to Capital Medical University and Beijing Ludaopei Hospital were retrospectively collected.The immunophenotype,EBV infection status and T-cell receptor(TCR)clonability of tumor cells were examined.The clinicopathological features were analyzed and related literatures were reviewed.Results There were 5 females and 3 males,aged 28 to 73 years,without B symptoms,lack of trauma and autoimmune diseases.Seven cases occurred in oral mucosa and one in anal canal mucosa.Submucosal nodules with ulcerations were presented in all cases except one,which only submucosal nodule.Morphologically,there was different distribution of allotypic lymphocytes in inflammatory background.Four cases showed“kidney-shaped”,“embryonic”and“horseshoe-shaped”cells,and one case resembled Hodgkin and Reed/Sternberg(HRS)cells.Allotypic lymphocytes expressed CD3(7/8),CD4+/CD8-(7/8)and CD4-/CD8-(1/8).CD30 was uniformly strongly positive while ALK and CD56 were negative.In situ hybridization of EBER was negative in five cases(5/5).Clonal TCR gene rearrangement was positive in two cases.Four patients did not receive radiotherapy or chemotherapy.All the seven patients survived without disease except one died due to concurrent leukopenia.Conclusions pmCD30+TLPD had a broad morphological spectrum and could be easily confused with primary cutaneous CD30+TLPD and systemic ALK-negative anaplastic large cell lymphoma involving mucosa,which may lead to misdiagnosis.Although the majority of the cases had a favorable prognosis,a few cases relapsed or progressed to lymphoma.

关 键 词：淋巴瘤 大细胞 间变性 抗原 CD30 淋巴组织增殖性疾病 黏膜 

分 类 号：R733[医药卫生—肿瘤]