一个罕见的流体过载相关的大型B细胞淋巴瘤病例和复发时的抗原损失
Xue Yan1, Bin Chen2, Hongxia Jing1
1Department of Hematology, The Affiliated Jiangning Hospital With Nanjing Medical University, Hushan Road, Jiangning District, Nanjing, 211100, Jiangsu, China.
Journal of hematopathology
|January 4, 2024
概括
流体过载相关的大B细胞淋巴瘤 (FO-LBCL) 是一种新分类的淋巴瘤. 这种病例突出了FO-LBCL与复发时的抗原损失,与初级溢出淋巴瘤 (PEL) 相比,显示出有利的预后.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 病理学 病理学 病理学
背景情况:
- 流体过载相关的大B细胞淋巴瘤 (FO-LBCL) 在世卫组织血液淋巴瘤分类 (WHO-HAEM5) 中得到认可.
- 区分FO-LBCL和原发性输液淋巴瘤 (PEL) 对于预后和治疗至关重要.
- FO-LBCL的特点是,通常比PEL更有利的结果.
研究的目的:
- 在复发期间呈现FO-LBCL与部分B细胞抗原损失的异常病例.
- 审查FO-LBCL的特征,治疗和预后,以改善理解.
- 根据临床和分子发现,区分FO-LBCL与PEL.
主要方法:
- 通过免疫组织化学和流细胞测量进行免疫型定型.
- 免疫球蛋白基因重新排列的分析 (BIOMED-2多重原料).
- 检测MYC,BCL2,BCL6基因重组 (FISH) 的情况.
- 使用下一代测序 (NGS) 对无细胞DNA (cfDNA) 的体质突变评估.
主要成果:
- 最初的诊断是结核性肺炎,后来修订为与HHV8无关的PEL类淋巴瘤.
- 复发显示出一种独特的免疫表型,部分B细胞抗原损失 (例如CD20阴性),但相同的IGH重组.
- 在CfDNA分析中,发现了CD79B,MYD88,CCND3和DTX1.1中的突变.
- 根据WHO-HAEM5标准,该患者被诊断为FO-LBCL.
结论:
- 诊断FO-LBCL需要仔细区分与PEL.
- 本案符合世卫组织HAEM5和ICC对EBV/HHV8阴性初级输出性淋巴瘤的分类.
- 患有FO-LBCL的患者可能有更好的预后,正如在22个月的良好结果中所示,在这种情况下,除了肺部排水之外,没有进一步的治疗.
相关概念视频
B Cell Activation and Differentiation
1.7K
The adaptive immune response, a sophisticated defense mechanism, relies on the activation and differentiation of B lymphocytes, or B cells. These processes enable our bodies to mount a tailored response against specific pathogens such as bacteria, free virus particles, toxins, and parasites.
When naive B cells encounter a specific antigen that can bind to the B cell receptor (BCR) on their surface, they undergo sensitization to respond to the antigen's presence. Sensitization begins with...
When naive B cells encounter a specific antigen that can bind to the B cell receptor (BCR) on their surface, they undergo sensitization to respond to the antigen's presence. Sensitization begins with...
1.7K
Cells of the Adaptive Immune Response
990
The T and B lymphocytes of the adaptive immune system develop from common lymphoid progenitor cells in the bone marrow. These progenitors give rise to precursors that eventually develop into both T and B lymphocytes. As these precursors mature, they gain the ability to detect and respond to foreign antigens in the body, a process known as immunocompetence. Additionally, these precursors acquire self-tolerance, a process that ensures they do not react to self-antigens. This intricate system...
990
Special Features of Adaptive Immunity
824
The adaptive immune system, a crucial component of the overall immune response, offers a highly specialized defense against pathogens. It involves specific cell types and features, enabling it to combat infections effectively and efficiently.
The primary cell types involved in adaptive immunity are T cells and B cells. Each type has a unique role in defending the body against pathogens. T cells are responsible for cell-mediated immunity. They identify and eliminate infected cells directly,...
The primary cell types involved in adaptive immunity are T cells and B cells. Each type has a unique role in defending the body against pathogens. T cells are responsible for cell-mediated immunity. They identify and eliminate infected cells directly,...
824


