咽淋巴环套细胞淋巴瘤4例及文献复习

doi:10.6040/j.issn.1673-3770.0.2019.490

摘要/Abstract

摘要： 目的分析发生于咽淋巴环的套细胞淋巴瘤的临床特点。方法回顾性分析北京大学第一医院耳鼻咽喉头颈外科2016~2019年度收治的4例发生于咽淋巴环的套细胞淋巴瘤,并结合文献复习,总结分析其临床表现、组织病理学、免疫组化、细胞遗传学、治疗和预后。结果发生于咽淋巴环的套细胞淋巴瘤报道较少,主要发病部位为腭扁桃体、鼻咽部及舌根,早期症状并不特异,肿瘤较大时出现相应的阻塞症状,鼻咽部表现为单侧或者双侧鼻堵,腭扁桃体及舌根表现为咽部异物感、吞咽阻挡感、口内含物感及说话含糊,B组症状少见。查体可见鼻咽部、腭扁桃体或舌根隆起肿物,确诊依靠病理,典型的免疫学特征为:CD5+、CD19+、CD20+, CD22+、CD43+、CD10+、CD23-,CyclinDl+,BCL-6-。分子遗传学特征是检测到t(11;14)(q13;q32)/(IgH/CCND1)染色体易位。套细胞淋巴瘤(MCL)被认为是一种不可治愈的淋巴瘤类型,就诊时往往处于Ⅲ、Ⅳ期,易发生结外及骨髓侵犯,目前还没有统一的治疗方案,多数预后较差。结论咽淋巴环MCL作为临床少见疾病,早期症状并不特异,病理及分子遗传学是确诊的方法,目前治疗方案并不统一,利妥昔单抗联合化疗方案及伊布替尼等新药的应用能够改善患者的预后。

关键词: 咽淋巴环, 套细胞淋巴瘤, 非霍奇金淋巴瘤

Abstract: Objective To analyze the clinical characteristics of mantle cell lymphoma(MCL)of the Waldeyer's ring. Methods A retrospective study of 4 patients with MCL in the Waldeyer's ring, treated in the department of otolaryngology, head and neck surgery of Peking University First Hospital between 2016 and 2019 were used in this review. Their clinical manifestations, histopathology, immunohistochemistry, cytogenetics, the treatment used, and prognosis were summarized and analyzed in combination with a literature review. Results MCL involving the Waldeyer's ring is extremely rare. The palatine tonsil is the most frequently involved site, followed by the nasopharynx and tongue base. They generally present with asymptomatic soft tissue swelling and typically lack the systemic B symptoms. When the tumor is large, there are corresponding obstruction symptoms. For MCL in the nasopharynx, the symptoms are a unilateral or bilateral nasal obstruction with or without ear fullness. For MCL in the palatine tonsil or tongue base, the most common symptoms were a sensation of fullness of the throat, difficulty swallowing, and not having clear speech because of the tumor in the oral cavity. Examination revealed soft tissue swelling of the nasopharynx, palatine tonsil or tongue base. MCL was diagnosed by examining the affected tissue obtained from a biopsy of the lymph node, tissue, bone marrow, or blood phenotype. MCLs have distinct immunophenotype expressing pan B-cell markers(CD 19, 20, and 22). Generally, the T-cell marker CD5 is also expressed, giving this tumor a characteristic profile. The most sensitive and specific marker for MCL is CyclinD1, of which almost all cases are moderately to strongly positive. MCL is characterized by a genetic profile of t(11;14)(q13;q32)in nearly all cases that can be evaluated by conventional cytogenetics and fluorescencein situ hybridization. MCL patients frequently present at an advanced disease stage with extranodal involvement, resulting in poor clinical outcomes due to frequent relapse and the aggressive course. Treatment options for MCL vary widely, with poor prognosis. Conclusions MCL may rarely occur in the Waldeyer's ring. It may be diagnostically challenging to differentiate from other lymphomas that may occur in this area. However, it exhibits some distinctive histological and immunohistochemistry traits which can help distinguish it. Due to the poor overall prognosis, early identification would be important in providing individualized treatment. Rituximab combined with chemotherapy and the use of new drugs such as ibrutinib can improve the prognosis of patients.

Key words: Waldeyer's ring, Mantle cell lymphoma, Non-Hodgkin's lymphoma

中图分类号:

R766

文锋, 王冰洁, 扈希昊, 赵恩民. 咽淋巴环套细胞淋巴瘤4例及文献复习[J]. 山东大学耳鼻喉眼学报, 2020, 34(1): 115-120.

WEN Feng, WANG Bingjie, HU Xihao, ZHAO Enmin. Mantle cell lymphoma involving the Waldeyer's ring: a case series and literature review[J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2020, 34(1): 115-120.

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