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Primary Cutaneous Lymphomas: Update of WHO-EORTC Classification

By: Susan Reckling
Posted: Monday, February 4, 2019

A recent update of their classification of primary cutaneous lymphomas was released by the World Health Organization (WHO) and the European Organisation for Research and Treatment of Cancer (EORTC). This update, which builds on the 2005 WHO-EORTC consensus classification and incorporates recent progress in both diagnosis and treatment of these skin conditions, was published in the journal Blood.

“Genome-wide genetic studies have contributed to a better understanding of the molecular pathways involved in the pathogenesis of the different types of cutaneous lymphomas and resulted in the recognition of additional diagnostic and prognostic criteria and new potential therapeutic targets,” commented Rein Willemze, MD, of the Leiden University Medical Center, The Netherlands, and colleagues.

Here are a few highlights from this update:

  • Primary cutaneous acral CD8-positive T-cell lymphoma and Epstein Barr virus (EBV)-positive mucocutaneous ulcer have been added as new provisional entities, and a new section on the cutaneous forms of chronic active EBV disease has been included.
  • Modifications have been made in the sections on lymphomatoid papulosis, increasing the spectrum of histologic and genetic types, and primary cutaneous marginal zone lymphomas, recognizing two different subtypes.
  • The characteristic features of the new and modified entities as well as the results of recent molecular studies with diagnostic, prognostic, and/or therapeutic significance for the different types of primary cutaneous lymphomas have been reviewed.
  • Information on the frequency and survival of the different types of primary cutaneous lymphomas has been provided.

“In the past decades, a multidisciplinary approach with collaboration between pathologists, dermatologists, hematologists, and radiation oncologists has been crucial for defining new entities and classifications and is also the best guarantee for further progress in the diagnosis and treatment of patients with a cutaneous lymphoma,” the authors concluded.

Disclosure: Dr.Willemze is a member of Takeda’s scientific advisory board. The other authors reported no conflicts of interest.



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