title: Reappraisal of the provisional entity primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma: a series of 10 adult and pediatric patients and review of the literature.
abstract: BACKGROUND
  Primary cutaneous CD4(+) small/medium-sized pleomorphic T-cell lymphoma (PCSM-TCL) was defined as a provisional entity in the 2005 World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. A limited number of reports describe a generally indolent but heterogeneous condition.
  
  
  OBJECTIVE
  We reviewed the concept of PCSM-TCL when applied to our experience and to the published literature.
  
  
  METHODS
  We conducted a retrospective chart review and a PubMed search to identify all reported cases of PCSM-TCL from 2005 to 2010.
  
  
  RESULTS
  Ten patients, including 4 of the youngest described, were identified in our institution, and their clinical and pathologic features were analyzed. All had a benign clinical course. Ten reports of patients with PCSM-TCL were reviewed and commonalities were found within an otherwise variable spectrum of clinical presentation, pathology, and biologic behavior.
  
  
  CONCLUSIONS
  This study was retrospective, follow-up was short term in some cases, and data were limited in a number of published reports.
  
  
  CONCLUSIONS
  Our experience of 10 cases that met the diagnostic criteria of the provisional entity PCSM-TCL highlights its occurrence in children and further supports characterization of this condition as a clonal T-cell lymphoproliferative disorder with indolent behavior.
authors.affiliation: Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa.
authors.forename: Christian L
authors.initials: CL
authors.lastname: Baum
authors.affiliation: Department of Internal Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa.
authors.forename: Brian K
authors.initials: BK
authors.lastname: Link
authors.affiliation: Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, Iowa.
authors.forename: Vishala T
authors.initials: VT
authors.lastname: Neppalli
authors.affiliation: Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa; Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, Iowa.
authors.forename: Brian L
authors.initials: BL
authors.lastname: Swick
authors.affiliation: Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa; Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, Iowa. Electronic address: vincent-liu@uiowa.edu.
authors.forename: Vincent
authors.initials: V
authors.lastname: Liu
mesh_terms: D000293:Adolescent; D000328:Adult; D000369:Aged, 80 and over; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies