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

  • Ilske Oschlies
  • Rebecca L. King
  • Snjezana Dotlic
  • Santiago Montes-Moreno
  • Maurilio Ponzoni
  • Alexandra Traverse-Glehen
  • Maria Calaminici
  • Judith A. Ferry
  • German Ott
  • John R GoodladEmail author
Review and Perspectives
Part of the following topical collections:
  1. Extranodal Lymphoproliferations: Organotypic Diseases and Diagnostic Challenges


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.


Cutaneous lymphomas Primary cutaneous B cell lymphoma Primary cutaneous T cell lymphomas Primary cutaneous CD30-positive lymphoproliferative disorder Primary cutaneous CD8-positive lymphomas 



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

Author 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.

Compliance with ethical standards

All authors declare strict adherence to compliance with ethical standards.

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Ilske Oschlies
    • 1
  • Rebecca L. King
    • 2
  • Snjezana Dotlic
    • 3
  • Santiago Montes-Moreno
    • 4
  • Maurilio Ponzoni
    • 5
  • Alexandra Traverse-Glehen
    • 6
  • Maria Calaminici
    • 7
  • Judith A. Ferry
    • 8
  • German Ott
    • 9
  • John R Goodlad
    • 10
    Email author
  1. 1.Department of Pathology, Hematopathology Section and Lymph Node RegistryUniversity Hospitals Schleswig-Holstein, Christian-Albrecht-UniversityKielGermany
  2. 2.Division of HematopathologyMayo ClinicRochesterUSA
  3. 3.Department of Pathology and CytologyUniversity Hospital Centre ZagrebZagrebCroatia
  4. 4.Department of Anatomic Pathology, Translational Hematopathology LabHospital Universitario Marques de Valdecilla/IDIVALSantanderSpain
  5. 5.Ateneo Vita-Salute San Raffaele and Unit of Lymphoid MalignanciesSan Raffaele Scientific InstituteMilanItaly
  6. 6.Department of HematopathologyHospices Civils de Lyon/Université Lyon 1LyonFrance
  7. 7.Department of Cellular Pathology, SIHMDS, Barts Health NHS Trust and Centre for Haemato-OncologyBarts Cancer InstituteLondonUK
  8. 8.Department of PathologyMassachusetts General Hospital and Harvard Medical SchoolBostonUSA
  9. 9.Department of Clinical PathologyRobert-Bosch-KrankenhausStuttgartGermany
  10. 10.Department of PathologyQueen Elizabeth University HospitalGlasgowScotland

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