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This chapter provides a comprehensive overview of primary cutaneous T-cell lymphomas and lymphoproliferative diseases of low malignant potential, a group defined more by their favorable prognosis. Key entities are lymphomatoid papulosis, primary cutaneous anaplastic large-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma. It highlights the diagnostic challenges due to their potential to mimic more aggressive malignancies and their generally indolent clinical course. Specific disease presentations, histopathological findings, and genetic markers, such as DUSP22-IRF4 rearrangements, are detailed alongside controversies surrounding some entities' malignant potential. The chapter also explores practical diagnostic approaches and therapeutic strategies, emphasizing the importance of avoiding overtreatment while ensuring accurate diagnosis and management.

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Primary Cutaneous T-Cell Lymphomas and Lymphoproliferative Diseases of Low Malignant Potential

  • 章节

  • 发表日期：

    2025年

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  • Robert Gniadecki,
  • Minakshi Taparia,
  • Jean Deschenes,
  • Jori Hardin,
  • Etienne Mahe

摘要

This chapter provides a comprehensive overview of primary cutaneous T-cell lymphomas and lymphoproliferative diseases of low malignant potential, a group defined more by their favorable prognosis. Key entities are lymphomatoid papulosis, primary cutaneous anaplastic large-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma. It highlights the diagnostic challenges due to their potential to mimic more aggressive malignancies and their generally indolent clinical course. Specific disease presentations, histopathological findings, and genetic markers, such as DUSP22-IRF4 rearrangements, are detailed alongside controversies surrounding some entities' malignant potential. The chapter also explores practical diagnostic approaches and therapeutic strategies, emphasizing the importance of avoiding overtreatment while ensuring accurate diagnosis and management.