套细胞淋巴瘤多形性及母细胞亚型3例OA
Three cases of pleomorphic and blastoid variants of mantle cell lymphoma
目的:原发于脾脏的套细胞淋巴瘤(mantle cell lymphoma,MCL)较少见.本研究旨在探究母细胞样及多形性MCL亚型的临床病理特征、诊断及鉴别诊断要点,提高临床医师对这2种罕见侵袭性亚型的认识,避免误诊.方法:收集患者资料,结合影像学检查结果,分析相关临床特征.观察和分析光学显微镜下肿瘤细胞的病理学形态特点.采用免疫组织化学染色法和原位杂交法检测相关标志物的表达情况.结果:3例患者年龄范围60~68岁,2例男性、1例女性,符合MCL典型的发病年龄与性别分布特点.组织形态学方面,多形性亚型的肿瘤细胞体积较大,呈高度多形性,细胞核形态极不规则,核仁显著,核分裂象极易见,形态与弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)高度相似;2例母细胞样亚型的肿瘤细胞为中等大小,形态相对单一,染色质细腻,核仁不明显,呈弥漫实性片状生长,局部形成模糊结节样结构,并可见玻璃样变性血管,与淋巴母细胞淋巴瘤(lymphoblastic lymphoma,LBL)形态特征相近.免疫表型分析显示,3例均特征性表达B细胞标志物及细胞周期蛋白(cyclin)D1、性别决定区Y相关高迁移率族盒蛋白11(SRY-box transcription factor 11,SOX11),但其中2例母细胞样亚型的CD5呈阴性,为不典型免疫表型,多形性亚型初诊时CD5阳性,复发后呈部分阳性;3例的Ki-67增殖指数均显著升高,范围为60%~90%,符合侵袭性淋巴瘤的高度增殖生物学行为,且1例多形性亚型复发后Ki-67增殖指数较初诊时进一步升高.结论:MCL母细胞样及多形性亚型是具有独特临床病理特征的高度侵袭性亚型,临床表现无特异性,组织学形态易与DLBCL、LBL等其他B细胞淋巴瘤混淆,且母细胞样亚型易出现CD5阴性的不典型免疫表型,病理诊断极具挑战性.精准诊断无法依靠单一指标完成,需结合患者临床资料、影像学表现、组织形态学特征及免疫表型分析进行综合判断,其中cyclin D1与SOX11为核心诊断标志物,Ki-67增殖指数偏高是预后不良的关键参考依据.病理医师在面对形态类似DLBCL或LBL的病例时,应将MCL母细胞样及多形性亚型纳入鉴别诊断范围,降低误诊率.这对评估患者预后,改善患者生存质量具有至关重要的临床意义.
Objective:Primary splenic mantle cell lymphoma(MCL)is uncommon.This study aims to investigate the clinicopathological characteristics,diagnostic features,and differential diagnostic considerations of the blastoid and pleomorphic variants of MCL,thereby improving recognition of these 2 rare and aggressive subtypes and reducing the risk of misdiagnosis. Methods:Clinical data and imaging findings were collected and analyzed.Histopathological features of tumor cells were evaluated under light microscopy.Immunohistochemistry and in situ hybridization were performed to assess the expression of relevant biomarkers. Results:The patients ranged in age from 60 to 68 years,2 males and 1 female,consistent with the typical age and gender distribution of MCL.Morphologically,the pleomorphic variant was characterized by large tumor cells with marked pleomorphism,highly irregular nuclei,prominent nucleoli,and frequent mitotic figures,closely resembling diffuse large B-cell lymphoma(DLBCL).In contrast,the 2 blastoid variants consisted of medium-sized,relatively monomorphic tumor cells with finely dispersed chromatin and inconspicuous nucleoli.These tumors exhibited diffuse solid-sheet growth,with focal indistinct nodular structures and hyalinized vessels,resembling lymphoblastic lymphoma(LBL).Immunophenotypic analysis demonstrated expression of B-cell markers,cyclin D1,and SRY-box transcription factor 11(SOX11).However,CD5 expression was absent in the 2 blastoid variants,representing an atypical immunophenotype.The pleomorphic variant was CD5-positive at initial diagnosis but showed only partial positivity at relapse.The Ki-67 proliferation index was significantly elevated in all cases,ranging from 60%to 90%,consistent with the highly proliferative biological behavior of aggressive lymphomas.In the relapsed pleomorphic case,the Ki-67 index increased further compared with that at initial diagnosis. Conclusion:The blastoid and pleomorphic variants of MCL are highly aggressive subtypes with distinctive clinicopathological features.Their clinical manifestations are nonspecific,and their histological morphology may closely mimic other B-cell lymphomas,particularly DLBCL and LBL.In addition,blastoid MCL may exhibit an atypical CD5-negative immunophenotype,making pathological diagnosis particularly challenging.Accurate diagnosis requires comprehensive integration of clinical information,imaging findings,histomorphological characteristics,and immunophenotypic profiles rather than reliance on a single parameter.Cyclin D1 and SOX11 are core diagnostic markers,whereas a high Ki-67 proliferation index serves as an important indicator of poor prognosis.When encountering cases morphologically resembling DLBCL or LBL,pathologists should include blastoid and pleomorphic MCL in the differential diagnosis to reduce misdiagnosis.Such recognition is crucial for prognostic assessment and optimization of patient management.
苏翔宇;陈超婵;孙天祎;叶端阳;赵敏;谢闵
蚌埠医科大学研究生院,蚌埠 233030||华东师范大学附属芜湖医院(芜湖市第二人民医院)病理科,芜湖 241001蚌埠医科大学研究生院,蚌埠 233030||华东师范大学附属芜湖医院(芜湖市第二人民医院)病理科,芜湖 241001华东师范大学附属芜湖医院(芜湖市第二人民医院)病理科,芜湖 241001||皖南医学院研究生院,芜湖 241002华东师范大学附属芜湖医院(芜湖市第二人民医院)病理科,芜湖 241001||皖南医学院研究生院,芜湖 241002华东师范大学附属芜湖医院(芜湖市第二人民医院)血液内科,芜湖 241001华东师范大学附属芜湖医院(芜湖市第二人民医院)病理科,芜湖 241001
医药卫生
套细胞淋巴瘤母细胞样亚型多形性亚型鉴别诊断免疫组织化学cyclin D1SOX11
mantle cell lymphomablastoid variantpleomorphic variantdifferential diagnosisimmunohistochemistrycyclin D1SOX11
《临床与病理杂志》 2026 (4)
643-650,8
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