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快速现场细胞学评估辅助诊断右心房纤维蛋白血栓中纤维蛋白相关大B细胞淋巴瘤:一例报告

Rapid On-Site Cytologic Evaluation-Facilitated Diagnosis of Fibrin-Associated Large B-Cell Lymphoma in a Right Atrial Fibrin Thrombus: A Case Report.

作者信息

Oga Kazuki, Tagawa Chiyuki, Teramoto Yuki, Ooe Takuhito, Saeki Miho, Takeuchi Yasuhide, Hirata Masahiro, Haga Hironori

机构信息

Faculty of Medicine, Kyoto University, Kyoto, Japan.

Department of Diagnostic Pathology, Nara Medical University, Nara, Japan.

出版信息

Case Rep Oncol. 2025 Jun 13;18(1):965-971. doi: 10.1159/000546869. eCollection 2025 Jan-Dec.

Abstract

BACKGROUND

Fibrin-associated large B-cell lymphoma (FA-LBCL) is a rare extranodal lymphoma confined to fibrin deposits within cardiovascular structures and other anatomically restricted spaces. Due to its non-mass-forming nature and nonspecific clinical presentation, preoperative diagnosis remains challenging.

CASE PRESENTATION

A 47-year-old woman with a history of mitral valve repair and pacemaker implantation presented with a right atrial mass extending from the coronary sinus. A catheter-based biopsy with rapid on-site cytologic evaluation (ROSE) revealed scattered large atypical lymphoid cells. Histology confirmed FA-LBCL, composed of CD20-positive cells with a high Ki-67 index (∼90%) and negative Epstein-Barr virus (EBV)-encoded RNA. The disease was staged as IE, with no evidence of extracardiac involvement. Despite no myocardial invasion, the patient opted for six cycles of R-CHOP and remains disease-free 2 years post-diagnosis.

CONCLUSION

This case highlights the diagnostic utility of ROSE in FA-LBCL, particularly in guiding sample adequacy and expediting ancillary studies. Given the focal and sparse distribution of neoplastic cells, early recognition through ROSE may prevent misdiagnosis and unnecessary procedures. As EBV-negative FA-LBCL represents a distinct entity with evolving molecular insights, further research is warranted to delineate its pathogenesis and optimal management strategies.

摘要

背景

纤维蛋白相关大B细胞淋巴瘤(FA-LBCL)是一种罕见的结外淋巴瘤,局限于心血管结构内的纤维蛋白沉积物和其他解剖学受限空间。由于其非肿块形成的性质和非特异性临床表现,术前诊断仍然具有挑战性。

病例报告

一名有二尖瓣修复和起搏器植入史的47岁女性,出现从冠状窦延伸的右心房肿块。基于导管的活检及快速现场细胞学评估(ROSE)显示散在的大的非典型淋巴细胞。组织学证实为FA-LBCL,由CD20阳性细胞组成,Ki-67指数高(约90%),爱泼斯坦-巴尔病毒(EBV)编码RNA阴性。疾病分期为IE期,无心脏外受累证据。尽管没有心肌浸润,但患者选择了六个周期的R-CHOP方案,诊断后2年仍无疾病。

结论

本病例突出了ROSE在FA-LBCL中的诊断作用,特别是在指导样本充足性和加快辅助检查方面。鉴于肿瘤细胞的局灶性和稀疏分布,通过ROSE早期识别可防止误诊和不必要的检查。由于EBV阴性的FA-LBCL代表一个具有不断发展的分子见解的独特实体,有必要进一步研究以阐明其发病机制和最佳管理策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4094/12266701/4f99d05f9919/cro-2025-0018-0001-546869_F01.jpg

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