[Diagnostics of primary cutaneous lymphomas].

Area of Science:

• Dermatology
• Oncology
• Hematology

Background:

• Primary cutaneous lymphomas can be challenging to diagnose, especially in early stages.
• Sézary syndrome often presents diagnostic difficulties, mimicking reactive erythroderma.
• Accurate diagnosis is crucial for effective treatment of cutaneous lymphomas.

Purpose of the Study:

• To review the diagnostic challenges of Sézary syndrome.
• To summarize clinical heterogeneity and diagnostic criteria for Sézary syndrome.
• To discuss recent advancements in Sézary cell detection and potential new biomarkers.

Main Methods:

• Review of clinical, histological, and immunohistochemical features of Sézary syndrome.
• Analysis of data from European Organisation for Research and Treatment of Cancer (EORTC) multicenter studies.
• Evaluation of molecular and cytogenetic characterization of tumor cells and potential diagnostic markers.

Main Results:

• Sézary syndrome diagnosis is complicated by its heterogeneity and overlap with other conditions.
• Detection of Sézary cells in blood remains challenging despite advanced techniques.
• New markers (CD158k, MYC, MNT, DNM, TWIST1, EPHA4, PLS3) show potential for differentiating Sézary syndrome from reactive erythroderma.

Conclusions:

• Accurate diagnosis of Sézary syndrome requires a comprehensive approach integrating clinical, histological, and molecular data.
• Further research is needed to validate novel biomarkers for routine clinical application in Sézary syndrome diagnosis.
• Improved diagnostic tools are essential for timely and accurate management of Sézary syndrome.