ORIGINAL ARTICLE

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

Chalid Assaf¹, Sylke Gellrich¹, Sean Whittaker², Alistair Robson², Lorenzo Cerroni³, Cesare Massone³, Helmut Kerl³, Christian Rose⁴, Andreas Chott⁵, Sergio Chimenti⁶, Christian Hallermann⁷, Tony Petrella⁸, Janine Wechsler⁹, Martine Bagot⁹, Michael Hummel¹⁰, Katrin Bullani-Kerl¹¹, Marcel W Bekkenk¹², Werner Kempf¹³, Chris J L M Meijer¹⁴, Rein Willemze¹² and Wolfram Sterry¹

¹ Department of Dermatology, Charité, Berlin, Germany
² Skin Tumour Unit, St John’s Institute of Dermatology, St Thomas’ Hospital, London, UK
³ Department of Dermatology, Medical University of Graz, Austria
⁴ Department of Dermatology, University of Lübeck, Germany
⁵ Department of Pathology, Vienna General Hospital, Medical University of Vienna, Austria
⁶ Department of Dermatology, University of Rome, Italy
⁷ Department of Dermatology, Georg August University of Göttingen, Germany
⁸ Department of Pathology, Centre Hospitalo-Universitaire, Dijon, France
⁹ Department of Pathology and Dermatology, Hôpital Henri Mondor, Creteil, France
¹⁰ Institute of Pathology, Charité, Berlin
¹¹ Department of Dermatology, University Hospital of Geneva, Switzerland
¹² Department of Dermatology, Leiden University, Medical Center, the Netherlands
¹³ Department of Dermatology, University Hospital of Zurich, Switzerland
¹⁴ Department of Pathology, Vrije Universiteit Medical Center, Amsterdam, the Netherlands

Correspondence to:
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

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.

Abbreviations: 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

Keywords: cutaneous lymphoma; CD56; extranodal NK/T-cell lymphoma; haematodermic neoplasm; CD123

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