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Aggressive cutaneous T-cell lymphomas.

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  • 1Ackerman Academy of Dermatopathology, 145 East 32nd, St 10th floor, New York, 10016, NY, USA.

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Prognosis for Cutaneous T cell lymphomas (CTCLs) depends on clinical, histopathologic, and immunophenotypic features. This review focuses on aggressive non-mycosis fungoides (MF) subtypes and aggressive MF presentations.

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Adult T-cell leukemia/lymphomaBerti's lymphomaCutaneous gamma delta T cell lymphoma

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Area of Science:

  • Dermatology
  • Oncology
  • Hematology

Background:

  • Cutaneous T cell lymphomas (CTCLs) are a diverse group of primary cutaneous lymphomas.
  • Prognosis is influenced by clinical, histopathologic, and immunophenotypic characteristics.
  • The WHO-EORTC classification categorizes primary cutaneous lymphomas.

Purpose of the Study:

  • To review aggressive non-mycosis fungoides (MF) subtypes of CTCL.
  • To discuss potentially aggressive presentations of MF.
  • To highlight diagnostic challenges, including distinguishing aggressive CTCL from benign lymphoproliferative disorders (LPD).

Main Methods:

  • Literature review focusing on non-MF aggressive CTCL subtypes.
  • Analysis of histopathologic, immunophenotypic, and clinical features of aggressive CTCL.
  • Discussion of differential diagnoses, including lymphomatoid papulosis (LyP).

Main Results:

  • Certain CTCL subtypes are associated with poor therapeutic response and short survival.
  • Aggressive subtypes can be identified by specific histologic, immunophenotypic, and clinical findings.
  • Benign LPDs like LyP can mimic aggressive lymphoma on biopsy.

Conclusions:

  • Predicting aggressive CTCL course can be challenging due to overlapping features with benign conditions and variable lymphoma behavior.
  • Accurate diagnosis requires careful integration of clinical, histopathologic, and immunophenotypic data.
  • Understanding aggressive CTCL subtypes is crucial for appropriate patient management and treatment planning.