title: Clinicopathological features and prognostic significance of CXCL12 in blastic plasmacytoid dendritic cell neoplasm.
abstract: BACKGROUND
  Blastic plasmacytoid dendritic cell neoplasm (BPDC) is a rare hematologic neoplasm, which almost always involves the skin and shows poor prognosis.
  
  
  OBJECTIVE
  The aim of our study was to enhance BPDC diagnosis and indications for prognosis.
  
  
  METHODS
  This study involved 26 patients with BPDC. To investigate the histogenesis of BPDC, we reviewed the clinical features and stained markers of various hematopoietic lineages, chemokines, and their receptors.
  
  
  RESULTS
  Bone-marrow infiltration was detected in 13 of the 19 cases examined and leukemic changes in 18. Complete remission was achieved in 14 cases, but more than half of the patients showed recurrence within a short time, and 14 patients died of the disease after 1 to 25 months (mean 8.5 months). Positivity for CD123 was detected in 18 of 24 cases and for T-cell leukemia 1 in 18 of 22 cases. Of the chemokines and their receptors, 8 of 15 skin biopsy specimens proved to be positive for CXCL12. Leukemic change subsequent to skin lesions occurred in 7 of 8 CXCL12-positive cases (87.5%) and in 3 of 6 CXCL12-negative cases (50%). Seven of the 8 CXCL12-positive patients (87.5%) and two of the 6 CXCL12-negative patients (33.3%) have died, whereas one of 8 CXCL12-positive patients (12.5%) and 4 of 6 CXCL12-negative patients (66.7%) remain alive.
  
  
  CONCLUSIONS
  The number of patients was limited.
  
  
  CONCLUSIONS
  We speculate that the presence of CXCL12-positive cells in the skin may be associated with leukemic change and a poor prognosis.
authors.affiliation: Department of Dermatology, Kurume University School of Medicine, Kurume, Japan.
authors.forename: Keiko
authors.initials: K
authors.lastname: Hashikawa
authors.forename: Daisuke
authors.initials: D
authors.lastname: Niino
authors.forename: Shinichiro
authors.initials: S
authors.lastname: Yasumoto
authors.forename: Takekuni
authors.initials: T
authors.lastname: Nakama
authors.forename: Junichi
authors.initials: J
authors.lastname: Kiyasu
authors.forename: Kensaku
authors.initials: K
authors.lastname: Sato
authors.forename: Yoshizo
authors.initials: Y
authors.lastname: Kimura
authors.forename: Masanori
authors.initials: M
authors.lastname: Takeuchi
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Sugita
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.forename: Koichi
authors.initials: K
authors.lastname: Ohshima
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D054377:Chemokine CXCL12 / Q000032:analysis*; D002675:Child, Preschool; D003713:Dendritic Cells / Q000276:immunology / Q000473:pathology*; D005260:Female; D019337:Hematologic Neoplasms / Q000401:mortality / Q000473:pathology; D006801:Humans; D007564:Japan / Q000453:epidemiology; D016410:Lymphoma, T-Cell, Cutaneous / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000401:mortality / Q000473:pathology*
chemical_list: D054377:Chemokine CXCL12