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Primary cutaneous CD30(+) lymphoproliferative disorders.

Iris Wieser1,2, Michael T Tetzlaff3, Carlos A Torres Cabala4

  • 1Department of Dermatology, The University of Texas, MD Anderson Cancer Center, Houston Texas, U.S.A.

Journal Der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
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Primary cutaneous CD30(+) lymphoproliferative disorders, including lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large T-cell lymphoma (cALCL), share overlapping features. Distinguishing these CTCL spectrum disorders requires careful clinicopathological correlation to ensure appropriate treatment.

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Area of Science:

  • Dermatology
  • Oncology
  • Hematopathology

Background:

  • Primary cutaneous CD30(+) lymphoproliferative disorders are the second most frequent cutaneous T-cell lymphomas (CTCL).
  • These disorders encompass lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large T-cell lymphoma (cALCL), sharing overlapping clinical, histopathological, and molecular characteristics.
  • Mycosis fungoides (MF), the most common CTCL, is distinct but can present with overlapping features or LyP-like lesions, necessitating careful differentiation.

Purpose of the Study:

  • To elucidate the spectrum of primary cutaneous CD30(+) lymphoproliferative disorders.
  • To highlight the importance of clinicopathological correlation in differentiating LyP from cALCL.
  • To address diagnostic challenges posed by overlapping features with Mycosis Fungoides and transformed MF.

Main Methods:

  • Review of clinical, histopathological, and molecular features of LyP and cALCL.
  • Analysis of disease coexistence and progression patterns.
  • Comparison with Mycosis Fungoides and transformed Mycosis Fungoides presenting with CD30 expression.

Main Results:

  • LyP and cALCL represent a spectrum of cutaneous CD30(+) lymphoproliferative disorders.
  • Clinicopathological correlation is crucial for distinguishing LyP from cALCL.
  • Coexistence and evolution between LyP and cALCL can occur; MF can mimic or coexist with LyP.

Conclusions:

  • Accurate diagnosis within the spectrum of cutaneous CD30(+) lymphoproliferative disorders is vital.
  • Careful evaluation is required to differentiate these entities from Mycosis Fungoides, especially in cases of transformed MF with CD30 expression.
  • Correct diagnosis ensures appropriate therapeutic strategies for patients with these lymphomas.