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CD56-positive haematological neoplasms of the skin: a multicentre study of the Cutaneous Lymphoma Project Group of the European Organisation for Research and Treatment of Cancer
  1. Chalid Assaf1,
  2. Sylke Gellrich1,
  3. Sean Whittaker2,
  4. Alistair Robson2,
  5. Lorenzo Cerroni3,
  6. Cesare Massone3,
  7. Helmut Kerl3,
  8. Christian Rose4,
  9. Andreas Chott5,
  10. Sergio Chimenti6,
  11. Christian Hallermann7,
  12. Tony Petrella8,
  13. Janine Wechsler9,
  14. Martine Bagot9,
  15. Michael Hummel10,
  16. Katrin Bullani-Kerl11,
  17. Marcel W Bekkenk12,
  18. Werner Kempf13,
  19. Chris J L M Meijer14,
  20. Rein Willemze12,
  21. Wolfram Sterry1
  1. 1Department of Dermatology, Charité, Berlin, Germany
  2. 2Skin Tumour Unit, St John’s Institute of Dermatology, St Thomas’ Hospital, London, UK
  3. 3Department of Dermatology, Medical University of Graz, Austria
  4. 4Department of Dermatology, University of Lübeck, Germany
  5. 5Department of Pathology, Vienna General Hospital, Medical University of Vienna, Austria
  6. 6Department of Dermatology, University of Rome, Italy
  7. 7Department of Dermatology, Georg August University of Göttingen, Germany
  8. 8Department of Pathology, Centre Hospitalo-Universitaire, Dijon, France
  9. 9Department of Pathology and Dermatology, Hôpital Henri Mondor, Creteil, France
  10. 10Institute of Pathology, Charité, Berlin
  11. 11Department of Dermatology, University Hospital of Geneva, Switzerland
  12. 12Department of Dermatology, Leiden University, Medical Center, the Netherlands
  13. 13Department of Dermatology, University Hospital of Zurich, Switzerland
  14. 14Department of Pathology, Vrije Universiteit Medical Center, Amsterdam, the Netherlands
  1. Correspondence to:
    Dr Chalid Assaf
    Department of Dermatology and Allergy, Skin Cancer Center Charité, Charité Universitätsmedizin Berlin, Fabeckstr 60–62, 14195 Berlin, Germany; chalid.assaf{at}charite.de

Abstract

Background: Cutaneous lymphomas expressing CD56, a neural cell adhesion molecule, are characterised in most cases by a highly aggressive clinical course and a poor prognosis. However, prognostic subsets within the CD56+ group have been difficult to identify due to the lack of uniform clinicopathological and immunophenotypical criteria.

Methods: A multicentre study was conducted by the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer to define prognostic parameters and establish diagnostic and therapeutic guidelines for CD56+ haematological neoplasms presenting primarily in the skin.

Results: Four different subtypes of lymphoproliferations with CD56 expression were identified: (1) haematodermic neoplasm; (2) skin infiltration as the first manifestation of CD56+ acute myeloid leukaemia; (3) nasal-type extranodal natural killer/T-cell lymphoma; and (4) “classical” cases of cutaneous T-cell lymphoma (CTCL) with co-expression of the CD56 molecule. Patients in the first three groups had a poor outcome (93% died) with a median survival rate of 11 months (95% CI 2–72 months), whereas all patients with CD56+ CTCL were alive at the last follow-up.

Conclusion: Results show that CD56+ cutaneous lymphoproliferative disorders, with the exception of CD56+ CTCL have a very poor prognosis. It is therefore clinically important to separate CD56+ CTCL from the remaining CD56+ haematological disorders.

  • AML, acute myelomonocytic leukaemia
  • CTCL, cutaneous T cell lymphoma
  • EBV, Epstein-Barr virus
  • EORTC, European Organisation for Research and Treatment of Cancer
  • NK, natural killer
  • pDC, plasmacytoid dendritic cells
  • TCR, T-cell receptor
  • cutaneous lymphoma
  • CD56
  • extranodal NK/T-cell lymphoma
  • haematodermic neoplasm
  • CD123

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Footnotes

  • Published Online First 3 October 2006

  • Competing interests: None declared.

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