title: 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.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Dermatology, Charité, Berlin, Germany. chalid.assaf@charite.de
authors.forename: Chalid
authors.initials: C
authors.lastname: Assaf
authors.forename: Sylke
authors.initials: S
authors.lastname: Gellrich
authors.forename: Sean
authors.initials: S
authors.lastname: Whittaker
authors.forename: Alistair
authors.initials: A
authors.lastname: Robson
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cerroni
authors.forename: Cesare
authors.initials: C
authors.lastname: Massone
authors.forename: Helmut
authors.initials: H
authors.lastname: Kerl
authors.forename: Christian
authors.initials: C
authors.lastname: Rose
authors.forename: Andreas
authors.initials: A
authors.lastname: Chott
authors.forename: Sergio
authors.initials: S
authors.lastname: Chimenti
authors.forename: Christian
authors.initials: C
authors.lastname: Hallermann
authors.forename: Tony
authors.initials: T
authors.lastname: Petrella
authors.forename: Janine
authors.initials: J
authors.lastname: Wechsler
authors.forename: Martine
authors.initials: M
authors.lastname: Bagot
authors.forename: Michael
authors.initials: M
authors.lastname: Hummel
authors.forename: Katrin
authors.initials: K
authors.lastname: Bullani-Kerl
authors.forename: Marcel W
authors.initials: MW
authors.lastname: Bekkenk
authors.forename: Werner
authors.initials: W
authors.lastname: Kempf
authors.forename: Chris J L M
authors.initials: CJ
authors.lastname: Meijer
authors.forename: Rein
authors.initials: R
authors.lastname: Willemze
authors.forename: Wolfram
authors.initials: W
authors.lastname: Sterry
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis*; D019002:CD56 Antigen / Q000032:analysis*; D002648:Child; D005260:Female; D005838:Genotype; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D011379:Prognosis; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; D019002:CD56 Antigen