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Cutaneous lymphoproliferative disorders: Back to the future.
1Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
Journal of Cutaneous Pathology
|March 19, 2024
Summary
Recent classifications re-evaluate low-grade cutaneous lymphomas. Experts now recognize primary cutaneous CD4+ small/medium T-cell lymphoma (PCSM-TCL) and primary cutaneous marginal zone lymphoma (PCMZL) as lymphoproliferative disorders (LPDs) due to their indolent behavior and excellent prognosis.
Area of Science:
- Dermatology and Hematopathology
- Oncology and Lymphoid Neoplasms
- Immunohistochemistry and Molecular Diagnostics
Background:
- Immunohistochemistry and clonality analyses in the 1980s redefined cutaneous lymphomas.
- Criteria for T-cell and B-cell proliferations led to reclassification of benign conditions as lymphomas.
- Growing evidence questions the specificity of these markers for malignancy.
Purpose of the Study:
- To review the evolving terminology of cutaneous lymphomas.
- To discuss the rationale behind reclassifying certain lymphomas as lymphoproliferative disorders (LPDs).
- To analyze the clinical implications of this reclassification.
Main Methods:
- Review of historical diagnostic criteria and classifications.
- Analysis of recent studies on low-grade cutaneous lymphomas.
- Comparison of the WHO (2022) and International Consensus Classification (ICC, 2022) approaches.
Main Results:
- Low-grade cutaneous T-cell lymphoma (CTCL) and cutaneous B-cell lymphoma (CBCL) exhibit indolent behavior and excellent prognosis.
- Recent classifications (WHO 2022, ICC 2022) have downgraded PCSM-TCL and related conditions to LPDs.
- Terminology for primary cutaneous marginal zone lymphoma (PCMZL) differs between the WHO and ICC classifications.
Conclusions:
- The diagnostic landscape for cutaneous lymphoid proliferations is dynamic.
- Reclassification to LPD reflects a better understanding of the biology and clinical behavior of these conditions.
- Understanding these terminology shifts is crucial for accurate diagnosis and patient management.
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