背景:由于大多数T细胞淋巴瘤缺乏CD20表达,表现出异常CD20表达的真菌病(MF)病例非常罕见。
目的:我们的目标是全面评估临床,组织病理学,7例CD20阳性MF患者的预后特征。
方法:这项回顾性研究检查了7例CD20异常表达的MF。该研究提供了人口统计的细节,临床特征,组织病理学和治疗结果。关键时间点包括MF的初始诊断,CD20表达的检测和随访,平均随访46个月。
结果:异常CD20阳性MF的诊断平均年龄为58.6岁,首次MF诊断后约5.6年。CD20检测后,患者表现为晚期疾病阶段,需要化疗等治疗,Brentuximabvedotin,和异基因造血干细胞移植。四名病人死于淋巴瘤,平均生存时间为52个月。
结论:MF中CD20异常表达罕见,但提示进展过程与不良预后相关。这通常需要全身化疗,在某些情况下,异基因造血干细胞移植。这项研究提供了对临床属性的重要见解,疾病进展,以及CD20表达异常的MF患者的治疗选择。需要进一步的研究来验证新兴疗法的有效性,并增强我们对这一独特MF亚组特有的潜在机制和预后决定因素的理解。
BACKGROUND: As the majority of T-cell lymphomas lack CD20 expression, cases of mycosis fungoides (MF) exhibiting aberrant CD20 expression are exceedingly uncommon.
OBJECTIVE: We aim to comprehensively evaluate the clinical, histopathological, and prognostic features of 7 patients diagnosed with CD20-positive MF.
METHODS: This retrospective
study examines seven cases of MF with aberrant CD20 expression. The
study provides details of demographics, clinical features, histopathology and treatment outcomes. Key time points include initial diagnosis of MF, detection of CD20 expression and follow-up, with a mean follow-up of 46 months.
RESULTS: Aberrant CD20-positive MF was diagnosed at an average age of 58.6 years, approximately 5.6 years after first MF diagnosis. Following CD20 detection, patients presented with advanced disease stages, requiring treatments such as chemotherapy, brentuximab vedotin, and allogeneic hematopoietic stem cell transplantation. Four patients died from lymphoma, with an average survival time of 52 months.
CONCLUSIONS: Aberrant CD20 expression in MF is rare but indicates a progressive course associated with poor prognosis. This often requires systemic chemotherapy and, in certain instances, allogeneic hematopoietic stem cell transplantation. This
study provides important insights into the clinical attributes, disease progression, and treatment options for MF patients with aberrant CD20 expression. Further research is necessary to validate the effectiveness of emerging therapies and enhance our understanding of the underlying mechanisms and prognostic determinants specific to this unique MF subgroup.