Leiden University Medical Center, Leiden, the Netherlands.
Department of Dermatology, University Medical Centre Mannheim, University of Heidelberg, Mannheim, Germany.
Br J Dermatol. 2021 Jul;185(1):19-25. doi: 10.1111/bjd.19669. Epub 2021 Feb 1.
Mycosis fungoides (MF) and Sézary syndrome (SS) are the best-studied subtypes of cutaneous T-cell lymphoma. The level of blood tumour burden in patients is important for diagnosis, disease staging, prognosis and management, as well as assessing treatment response. Until recently, the assessment of blood involvement was made using manual counts of morphologically atypical T cells (Sézary cells), but this approach may be subjective, and is affected by interobserver variability. Objective and consistent approaches to accurately quantifying blood involvement are required to ensure appropriate stage-related management of patients and to improve our understanding of the prognostic implications of blood tumour burden in these diseases. While assessment of blood involvement is common in SS and advanced-stage MF, an improved understanding of the implications of blood involvement at early disease stages could help identify patients more likely to progress to late-stage disease, and hence guide treatment decisions and frequency of follow-up assessment, ultimately improving patient outcomes. This concise review discusses the development of flow cytometry-based classifications for assessing blood involvement in MF and SS, and summarizes current recommendations for blood classification and assessment of blood response to treatment.
蕈样肉芽肿(MF)和赛泽里综合征(SS)是研究得最好的皮肤 T 细胞淋巴瘤亚型。患者血液肿瘤负担水平对诊断、疾病分期、预后和管理以及评估治疗反应都很重要。直到最近,血液受累的评估都是通过形态异常的 T 细胞(Sézary 细胞)的人工计数来进行的,但这种方法可能存在主观性,并且受到观察者间变异性的影响。需要客观和一致的方法来准确量化血液受累程度,以确保对患者进行适当的与疾病分期相关的管理,并提高我们对这些疾病中血液肿瘤负担预后意义的理解。虽然 SS 和晚期 MF 中常见评估血液受累情况,但对早期疾病阶段血液受累的意义有更深入的了解可能有助于识别更有可能进展为晚期疾病的患者,从而指导治疗决策和随访评估的频率,最终改善患者的预后。本简明综述讨论了基于流式细胞术评估 MF 和 SS 血液受累的分类方法的发展,并总结了目前关于血液分类和评估血液对治疗反应的建议。