Wang X Y, Li M, Zhao Y X, Wang L F, Li D J, Xu Z G, Kong L F
Department of Pathology, Henan Provincial People's Hospital/People's Hospital of Zhengzhou University, Zhengzhou 450003, China.
Department of Pathology, Peking University School of Basic Medical Sciences, Beijing 100191 China.
Zhonghua Bing Li Xue Za Zhi. 2022 Apr 8;51(4):301-306. doi: 10.3760/cma.j.cn112151-20211220-00915.
To investigate the clinicopathological features as well as BRAF V600E and MYD88 L265P mutation status of nodal marginal zone B cell lymphoma (NMZL). Thirty-two cases of NMZL were diagnosed from September 2009 to February 2021 at the Henan Provincial People's Hospital and Peking University School of Basic Medical Sciences. The clinicopathologic characteristics were obtained and analyzed. BRAF V600E and MYD88 L265P mutation status were identified using PCR and Sanger sequencing, respectively. There were 20 males and 12 females patients with a median age of 69 years (ranging 36-82 years). The most prevalent clinical manifestation was multiple lymph nodes enlargement in head and neck (22/32, 68.8%), followed by inguinal (12/32, 37.5%), axillary (11/32, 34.4%), mediastinum (5/32, 15.6%) and retroperitoneal lymph nodes (4/32, 12.5%). Most of the patients were in Ann Arbor stage Ⅰ/Ⅱ (21 cases). The morphologic features included diffuse (24/32, 75.0%), nodular (5/32, 15.6%), interfollicular (2/32,6.3%) and perifollicular (1/32,3.1%) types. The tumor cells showed monocyte-like, centrocyte-like, small lymphocyte-like and plasma cell-like differentiation. Immunophenotyping revealed diffuse expression of CD20 in all tumor cells, whereas CD43 (11/32, 34.4%), bcl-2 (20/32, 62.5%), MNDA (13/32, 40.6%) and CD5 (2/32, 6.3%) were partially expressed. Ki-67 proliferation index varied from 10% to 40%. BRAF V600E mutation was found in two cases (2/32, 6.3%), but MYD88 L265P mutation was not detected. Eighteen patients survived and three died at the end of follow-up period which ranged 6 to 110 months. The morphologic features of NMZL varies across individuals, it should be differentiated from various B-cell lymphomas; however immunological biomarkers with high specificity for NMZL are still lacking. No MYD88 L265P mutation is found in NMZL. Some cases may harbor BRAF V600E mutation and yet the prevalence remains indeterminate; further researches are warranted.
探讨淋巴结边缘区B细胞淋巴瘤（NMZL）的临床病理特征以及BRAF V600E和MYD88 L265P突变状态。2009年9月至2021年2月期间，在河南省人民医院和北京大学基础医学院诊断出32例NMZL。获取并分析其临床病理特征。分别采用聚合酶链反应（PCR）和桑格测序法鉴定BRAF V600E和MYD88 L265P突变状态。患者中男性20例，女性12例，中位年龄69岁（范围36 - 82岁）。最常见的临床表现是头颈部多发淋巴结肿大（22/32，68.8%），其次是腹股沟（12/32，37.5%）、腋窝（11/32，34.4%）、纵隔（5/32，15.6%）和腹膜后淋巴结（4/32，12.5%）。大多数患者处于Ann Arbor分期Ⅰ/Ⅱ期（21例）。形态学特征包括弥漫型（24/32，75.0%）、结节型（5/32，15.6%）、滤泡间型（2/32，6.3%）和滤泡周围型（1/32，3.1%）。肿瘤细胞表现出单核细胞样、中心细胞样、小淋巴细胞样和浆细胞样分化。免疫表型分析显示所有肿瘤细胞中CD20弥漫性表达，而CD43（11/32，34.4%）、bcl - 2（20/32，62.5%）、MNDA（13/32，40.6%）和CD5（2/32，6.3%）部分表达。Ki - 67增殖指数在10%至40%之间。发现2例（2/32，6.3%）存在BRAF V600E突变，但未检测到MYD88 L265P突变。随访期为6至110个月，随访结束时18例患者存活，3例死亡。NMZL的形态学特征因人而异，应与各种B细胞淋巴瘤相鉴别；然而，仍缺乏对NMZL具有高特异性的免疫生物标志物。在NMZL中未发现MYD88 L265P突变。部分病例可能存在BRAF V600E突变，但其发生率仍不确定，有必要进一步研究。
