title: Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients.
abstract: BACKGROUND
  Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease characterized by malignant proliferation of a contingent blastic plasmacytoid dendritic cell. This rare entity is recognized mostly by cutaneous spreading, or not having a leukaemic component. The prognosis is very poor.
  
  
  OBJECTIVE
  To study a large cohort of 90 patients with BPDCN, to define additional symptoms to form a correct diagnosis earlier, and to manage such patients accordingly.
  
  
  METHODS
  We retrospectively reviewed BPDCN cases registered in the French Study Group on Cutaneous Lymphoma database between November 1995 and January 2012. Ninety patients were studied. Demographic data, clinical presentation, initial staging and outcome were recorded.
  
  
  RESULTS
  The group contained 62 male and 28 female patients (sex ratio 2·2). Their ages ranged from 8 to 103 years at the time of diagnosis (mean 67·2 years). Three major different clinical presentations were identified. Sixty-six patients (73%) presented with nodular lesions only, 11 patients (12%) with 'bruise-like' patches and 13 (14%) with disseminated lesions (patches and nodules). Mucosal lesions were seen in five patients (6%). The median survival in patients with BPDCN was 12 months.
  
  
  CONCLUSIONS
  We here distinguish three different clinical presentations of BPDCN. A nodular pattern is a more common feature than the originally reported 'bruise-like' pattern. Despite the fact that BPDCN may initially appear as a localized skin tumour, aggressive management including allogeneic bone marrow transplantation should be considered immediately, as it is currently the only option associated with long-term survival.
authors: Department of Dermatology, Cancer Research Center of Lyon, Claude Bernard Lyon 1 University, Hospices Civils de Lyon, Lyon, France.
authors: F
authors: F
authors: Julia
authors: T
authors: T
authors: Petrella
authors: M
authors: M
authors: Beylot-Barry
authors: M
authors: M
authors: Bagot
authors: D
authors: D
authors: Lipsker
authors: L
authors: L
authors: Machet
authors: P
authors: P
authors: Joly
authors: O
authors: O
authors: Dereure
authors: M
authors: M
authors: Wetterwald
authors: M
authors: M
authors: d'Incan
authors: F
authors: F
authors: Grange
authors: J
authors: J
authors: Cornillon
authors: G
authors: G
authors: Tertian
authors: E
authors: E
authors: Maubec
authors: P
authors: P
authors: Saiag
authors: S
authors: S
authors: Barete
authors: I
authors: I
authors: Templier
authors: F
authors: F
authors: Aubin
authors: S
authors: S
authors: Dalle
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D057210:Delayed Diagnosis; D003713:Dendritic Cells / Q000473:pathology*; D005260:Female; D019337:Hematologic Neoplasms / Q000401:mortality / Q000473:pathology*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009061:Mouth Mucosa / Q000473:pathology; D009062:Mouth Neoplasms / Q000401:mortality / Q000473:pathology*; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000401:mortality / Q000473:pathology*; D055815:Young Adult