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Alexander Scheu1, Saskia Maria Schnabl1, Daniel Patric Steiner1

  • 1Universitäts-Hautklinik Tübingen, Eberhard Karls Universität Tübingen.

Journal Der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
|March 12, 2021
PubMed
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Primary cutaneous lymphomas (PCL) often present with a diagnostic delay of several years. Phototherapy remains a primary treatment for early-stage mycosis fungoides, a common PCL subtype.

Area of Science:

  • Dermatology
  • Oncology
  • Hematology

Context:

  • Primary cutaneous lymphomas (PCL) exhibit distinct clinical behaviors and prognoses compared to systemic lymphomas of the same histological type.
  • Understanding PCL subtypes, diagnostic timelines, and treatment strategies is crucial for effective patient management.
  • This study analyzes a cohort of PCL patients to provide insights into their clinical characteristics and outcomes.

Purpose:

  • To investigate the distribution of PCL subtypes.
  • To determine the time from disease manifestation to diagnosis.
  • To evaluate the role of diagnostic procedures, secondary tumor occurrence, and treatment modalities in PCL patients.

Summary:

  • A retrospective analysis of 152 PCL patients revealed 105 with primary cutaneous T-cell lymphoma (CTCL) and 47 with primary cutaneous B-cell lymphoma (CBCL).

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  • The average time from symptom onset to diagnosis was four years, with mycosis fungoides (MF) being the most frequent diagnosis (47.4%).
  • First-line therapies included phototherapy (PUVA/UVB) or combination therapies, with Interferon-alpha plus PUVA as a common second-line treatment. Overall outcomes were favorable, with 45.2% achieving remission.

    • Impact:

    • Diagnostic delays for PCLs can be substantial, often spanning several years.
    • The utility of staging procedures in PCL management appears limited.
    • Phototherapy is a cornerstone treatment for early-stage mycosis fungoides, guiding therapeutic decisions in PCL care.