本文为回顾性研究,遵守《赫尔辛基宣言》,经过兰州大学第二医院伦理委员会批准,免除受试者知情同意,批准文号:2023A-169。患者女,60岁,因“右上肢麻木无力三个月,加重一周”于2018年5月至兰州大学第二医院就诊。专科检查:神志清,格拉斯...本文为回顾性研究,遵守《赫尔辛基宣言》,经过兰州大学第二医院伦理委员会批准,免除受试者知情同意,批准文号:2023A-169。患者女,60岁,因“右上肢麻木无力三个月,加重一周”于2018年5月至兰州大学第二医院就诊。专科检查:神志清,格拉斯哥昏迷评分(Glasgow Coma Scale,GCS)15分,查体合作,自动体位,言语清晰,记忆力、理解力、定向力检查未见异常。双侧瞳孔等大等圆,对光反射存在。四肢肌张力正常,四肢体肌力5级,四肢未见肌肉萎缩及震颤。右上肢麻木,以右手手指为著,其余四肢深浅感觉检查未见异常,双侧指鼻试验、跟膝胫试验正常。闭目难立征(−)。颈软无抵抗,双侧克氏征、布氏征均为阴性。实验室检查结果未见明显异常。展开更多
Aim: To determine (a) the expression of plasma cell related antigens in extran odal marginal zone B cell lymphomas (EMZL) of the ocular adnexa; and (b) the pro gnostic value of plasmacellular differentiation in these ...Aim: To determine (a) the expression of plasma cell related antigens in extran odal marginal zone B cell lymphomas (EMZL) of the ocular adnexa; and (b) the pro gnostic value of plasmacellular differentiation in these tumours. Methods: A con secutive case series of 136 ocular adnexal EMZL obtained from three ocular patho logy centres over 20 years was analysed retrospectively. An extensive immunohist ochemical panel, including the plasma cell related antigens VS38c, CD38, CD138, multiple myeloma oncogene-1-protein (MUM1/ IRF4), and CREB binding protein (CB P) was performed. EMZL were defined as “plasmacellular differentiated”on the b asis of morphological features, evidence of cytoplasmic immunoglobulin, negativi ty for BSAP/PAX5, and expression of at least one of the investigated plasma cell related antigens. Controls included normal or hyperplastic lymphatic tissues. D etailed clinical data were collected for most patients, and compared with the re sults of immunohistochemistry. The end points considered for statistical analysi s were development of local tumour recurrence, development of systemic disease, and lymphoma related death. Results: 57 (42%) of the 136 ocular adnexal EMZL sh owed a plasmacellular differentiation; 45 of these plasmacytoid cases were prima ry tumours. In contrast with most admixed normal plasma cells, which displayed c o-expression of MUM1/IRF4, Vs38c, CD38, CD138, and CBP, the plasmacellular diff erentiated EMZL tumour cells demonstrated co-expression of all five plasma cell related antigens in only six of 57 (11%) plasmacellular differentiated ocular adnexal EMZL. The most commonly expressed plasma cell related antigen was MUM1/I RF4, immunoreactivity being seen in 56/57 (98%) plasmacellular differentiated E MZL examined. Although the association of plasmacellular differentiation in prim ary ocular adnexal EMZL and disseminated disease was statistically significant o n univariate analysis (p=0.042), this was weaker on multivariate analysis. Concl usion: Plasmacellular differentiated tumour cells in EMZL demonstrate an aberran t immune profile for plasma cell related antigens when compared with normal plas ma cells. On multivariate analysis, plasmacellular differentiation in ocular adn exal EMZL was not significantly associated with local recurrence, the developmen t of systemic disease, or with lymphoma related death.展开更多
目的观察结节性淋巴细胞为主型霍奇金淋巴瘤(nodular lymphocyte-predominant Hodgkin lymphoma,NLPHL)伴少见免疫结构变异即富于T细胞/组织细胞大B细胞淋巴瘤(T-cell/histiocyte-rich large B cell lymphoma,THRLBL)样转化的NLPHL的临...目的观察结节性淋巴细胞为主型霍奇金淋巴瘤(nodular lymphocyte-predominant Hodgkin lymphoma,NLPHL)伴少见免疫结构变异即富于T细胞/组织细胞大B细胞淋巴瘤(T-cell/histiocyte-rich large B cell lymphoma,THRLBL)样转化的NLPHL的临床病理学特征,以提高对NLPHL免疫结构变异的认识、诊断及鉴别诊断。方法回顾性分析1例伴有THRLBL样转化的NLPHL的临床病理学特征及免疫表型。行EB病毒相关性和Ig/TCR基因克隆性检测,并复习相关文献。结果患者男性,58岁,腹股沟区无痛性淋巴结肿大。腹股沟淋巴结活检组织学观察可见淋巴结结构破坏,低倍镜下见浅染区和深染区交替分布,以浅染区为主,两种区域均可见散在分布的异型大细胞。免疫表型:大细胞一致强表达全B细胞标记(CD20、PAX5)、不表达CD30;CD21显示深染区内不规则滤泡树突细胞网结构,而浅染区内缺如。此外,两种结构背景细胞组成也存在明显差异。深染区背景细胞富于小B细胞,并可见PD1阳性细胞围绕大细胞形成花环样结构;浅染区背景细胞则以小T细胞和组织细胞为主,小B细胞基本缺如,且PD1阳性细胞量及强度均显著下降。EB病毒原位杂交检测两种结构内均无阳性细胞,Ig和TCR基因重排检测均未发现克隆性重排。结论伴有THRLBL样转化的NLPHL具有特殊形态学和免疫结构特征,易被误诊为原发性THRLBL,了解NLPHL免疫结构变异并结合细致全面的组织学观察和免疫组化检测有助于其诊断和鉴别诊断。展开更多
文摘本文为回顾性研究,遵守《赫尔辛基宣言》,经过兰州大学第二医院伦理委员会批准,免除受试者知情同意,批准文号:2023A-169。患者女,60岁,因“右上肢麻木无力三个月,加重一周”于2018年5月至兰州大学第二医院就诊。专科检查:神志清,格拉斯哥昏迷评分(Glasgow Coma Scale,GCS)15分,查体合作,自动体位,言语清晰,记忆力、理解力、定向力检查未见异常。双侧瞳孔等大等圆,对光反射存在。四肢肌张力正常,四肢体肌力5级,四肢未见肌肉萎缩及震颤。右上肢麻木,以右手手指为著,其余四肢深浅感觉检查未见异常,双侧指鼻试验、跟膝胫试验正常。闭目难立征(−)。颈软无抵抗,双侧克氏征、布氏征均为阴性。实验室检查结果未见明显异常。
文摘Aim: To determine (a) the expression of plasma cell related antigens in extran odal marginal zone B cell lymphomas (EMZL) of the ocular adnexa; and (b) the pro gnostic value of plasmacellular differentiation in these tumours. Methods: A con secutive case series of 136 ocular adnexal EMZL obtained from three ocular patho logy centres over 20 years was analysed retrospectively. An extensive immunohist ochemical panel, including the plasma cell related antigens VS38c, CD38, CD138, multiple myeloma oncogene-1-protein (MUM1/ IRF4), and CREB binding protein (CB P) was performed. EMZL were defined as “plasmacellular differentiated”on the b asis of morphological features, evidence of cytoplasmic immunoglobulin, negativi ty for BSAP/PAX5, and expression of at least one of the investigated plasma cell related antigens. Controls included normal or hyperplastic lymphatic tissues. D etailed clinical data were collected for most patients, and compared with the re sults of immunohistochemistry. The end points considered for statistical analysi s were development of local tumour recurrence, development of systemic disease, and lymphoma related death. Results: 57 (42%) of the 136 ocular adnexal EMZL sh owed a plasmacellular differentiation; 45 of these plasmacytoid cases were prima ry tumours. In contrast with most admixed normal plasma cells, which displayed c o-expression of MUM1/IRF4, Vs38c, CD38, CD138, and CBP, the plasmacellular diff erentiated EMZL tumour cells demonstrated co-expression of all five plasma cell related antigens in only six of 57 (11%) plasmacellular differentiated ocular adnexal EMZL. The most commonly expressed plasma cell related antigen was MUM1/I RF4, immunoreactivity being seen in 56/57 (98%) plasmacellular differentiated E MZL examined. Although the association of plasmacellular differentiation in prim ary ocular adnexal EMZL and disseminated disease was statistically significant o n univariate analysis (p=0.042), this was weaker on multivariate analysis. Concl usion: Plasmacellular differentiated tumour cells in EMZL demonstrate an aberran t immune profile for plasma cell related antigens when compared with normal plas ma cells. On multivariate analysis, plasmacellular differentiation in ocular adn exal EMZL was not significantly associated with local recurrence, the developmen t of systemic disease, or with lymphoma related death.
文摘目的观察结节性淋巴细胞为主型霍奇金淋巴瘤(nodular lymphocyte-predominant Hodgkin lymphoma,NLPHL)伴少见免疫结构变异即富于T细胞/组织细胞大B细胞淋巴瘤(T-cell/histiocyte-rich large B cell lymphoma,THRLBL)样转化的NLPHL的临床病理学特征,以提高对NLPHL免疫结构变异的认识、诊断及鉴别诊断。方法回顾性分析1例伴有THRLBL样转化的NLPHL的临床病理学特征及免疫表型。行EB病毒相关性和Ig/TCR基因克隆性检测,并复习相关文献。结果患者男性,58岁,腹股沟区无痛性淋巴结肿大。腹股沟淋巴结活检组织学观察可见淋巴结结构破坏,低倍镜下见浅染区和深染区交替分布,以浅染区为主,两种区域均可见散在分布的异型大细胞。免疫表型:大细胞一致强表达全B细胞标记(CD20、PAX5)、不表达CD30;CD21显示深染区内不规则滤泡树突细胞网结构,而浅染区内缺如。此外,两种结构背景细胞组成也存在明显差异。深染区背景细胞富于小B细胞,并可见PD1阳性细胞围绕大细胞形成花环样结构;浅染区背景细胞则以小T细胞和组织细胞为主,小B细胞基本缺如,且PD1阳性细胞量及强度均显著下降。EB病毒原位杂交检测两种结构内均无阳性细胞,Ig和TCR基因重排检测均未发现克隆性重排。结论伴有THRLBL样转化的NLPHL具有特殊形态学和免疫结构特征,易被误诊为原发性THRLBL,了解NLPHL免疫结构变异并结合细致全面的组织学观察和免疫组化检测有助于其诊断和鉴别诊断。
