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Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (pcCD8-AETCL), primary cutaneous γδ T-cell lymphoma (pcGDTCL), NK/T-lymphoma, and cutaneous peripheral T-cell lymphoma (not otherwise specified) comprise the clinical category of “aggressive cutaneous lymphomas.” Mortality in patients with extracutaneous involvement is high (>90% at 5 years). Patients presenting with skin-only disease seem to have a better prognosis. Clinical manifestations are heterogeneous (plaques, tumors, ulcers); the lesions can be solitary or multiple. There is no consensus regarding the optimal therapeutic approach. Allogeneic hematopoietic stem cell transplant is a promising option for eligible patients.

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Aggressive Cutaneous T-Cell and NK/T-Cell Lymphomas

  • 章节

  • 发表日期：

    2025年

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

摘要

Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (pcCD8-AETCL), primary cutaneous γδ T-cell lymphoma (pcGDTCL), NK/T-lymphoma, and cutaneous peripheral T-cell lymphoma (not otherwise specified) comprise the clinical category of “aggressive cutaneous lymphomas.” Mortality in patients with extracutaneous involvement is high (>90% at 5 years). Patients presenting with skin-only disease seem to have a better prognosis. Clinical manifestations are heterogeneous (plaques, tumors, ulcers); the lesions can be solitary or multiple. There is no consensus regarding the optimal therapeutic approach. Allogeneic hematopoietic stem cell transplant is a promising option for eligible patients.