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The clinico-pathological spectrum of primary cutaneous lymphoma other than mycosis fungoides/Sezary syndrome

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Abstract

The major aim of Session 1 of the 2018 European Association of Hematopathology/Society for Hematopathology Workshop was to collect examples of cutaneous lymphomas, excluding mycosis fungoides/Sezary syndrome, as defined in the current WHO classification of tumours of the haemetopoietic and lymphoid tissues. Overall 42 cases were submitted. These were considered in four main categories: primary cutaneous B cell lymphomas (12 cases), primary cutaneous T cell lymphomas/lymphoproliferations with CD8+/cytotoxic phenotype (12 cases), primary cutaneous CD30-positive lymphoproliferative disorders (15 cases) and primary cutaneous T cell lymphomas/leukaemias with CD4+ phenotype (4 cases). Using these cases as examples, we were able to present the full spectrum of cutaneous lymphoproliferations (excluding mycosis fungoides/Sezary syndrome), including examples of rare, provisional and new entities as listed in the 2017 update of the WHO classification. The findings are summarized in this report with emphasis on differential diagnostic considerations and the importance of clinico-pathological correlation for final subtyping. In presenting these findings we hope to raise awareness of this enigmatic group of neoplasms and to further our understanding of these rare disease entities.

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Acknowledgements

The authors thank all workshop participants for their case submissions and for use of images in this review.

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Authors and Affiliations

  1. Department of Pathology, Hematopathology Section and Lymph Node Registry, University Hospitals Schleswig-Holstein, Christian-Albrecht-University, Kiel, Germany

    Ilske Oschlies

  2. Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

    Rebecca L. King

  3. Department of Pathology and Cytology, University Hospital Centre Zagreb, Zagreb, Croatia

    Snjezana Dotlic

  4. Department of Anatomic Pathology, Translational Hematopathology Lab, Hospital Universitario Marques de Valdecilla/IDIVAL, Santander, Spain

    Santiago Montes-Moreno

  5. Ateneo Vita-Salute San Raffaele and Unit of Lymphoid Malignancies, San Raffaele Scientific Institute, Milan, Italy

    Maurilio Ponzoni

  6. Department of Hematopathology, Hospices Civils de Lyon/Université Lyon 1, Lyon, France

    Alexandra Traverse-Glehen

  7. Department of Cellular Pathology, SIHMDS, Barts Health NHS Trust and Centre for Haemato-Oncology, Barts Cancer Institute, London, UK

    Maria Calaminici

  8. Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Judith A. Ferry

  9. Department of Clinical Pathology, Robert-Bosch-Krankenhaus, Stuttgart, Germany

    German Ott

  10. Department of Pathology, Queen Elizabeth University Hospital, Level 3, Laboratory Medicine Building, 1345 Govan Rd, Glasgow, G51 4TF, Scotland

    John R Goodlad

Authors
  1. Ilske Oschlies
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  2. Rebecca L. King
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  4. Santiago Montes-Moreno
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  10. John R Goodlad
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Contributions

All authors conceived and designed the review; all authors participated into the collection, discussion and the final diagnostic work-up of the submitted cases. All authors wrote, edited and reviewed the manuscript; all authors gave their final approval for publication. I.O and J.G. take full responsibility to the work as a whole including the reported data from individual workshop cases.

Corresponding author

Correspondence to John R Goodlad.

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Oschlies, I., King, R.L., Dotlic, S. et al. The clinico-pathological spectrum of primary cutaneous lymphoma other than mycosis fungoides/Sezary syndrome. Virchows Arch 476, 683–699 (2020). https://doi.org/10.1007/s00428-019-02713-7

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