title: Cutaneous Richter syndrome: report of 3 cases from one institution.
abstract: BACKGROUND
  Richter syndrome (RS) is large-cell transformation of chronic lymphocytic leukemia (CLL). It commonly involves lymph nodes and bone marrow, but may rarely manifest in skin. Certain triggering factors, such as Epstein-Barr virus infection and p53 overexpression, have been implicated in the pathogenesis of RS. Here, we present 3 cases of cutaneous RS from our institution with a follow-up period of up to 8 years.
  
  
  OBJECTIVE
  We present a series of cutaneous RS from a single institution with the longest follow-up period (up to 8 years) to date.
  
  
  METHODS
  Clinical characteristics were collected and histopathological findings of skin biopsy specimens were analyzed.
  
  
  RESULTS
  All 3 patients had prior CLL and later developed cutaneous RS lesions. The mean age at the diagnosis of cutaneous RS was 67 years old. The time intervals between CLL and cutaneous RS were 3 to 8 years. Skin biopsy specimens demonstrated dermal nodular or perivascular infiltrates of large B cells, showing similar immunophenotypes to the lesional cells in the original CLL. Overexpression of p53 and positive stain for Epstein-Barr virus--encoded small RNA was found in one patient. One patient remained alive 8 years after the diagnosis whereas the other two died of the disease at 5 years and 3 weeks, respectively, after the onset of cutaneous RS.
  
  
  CONCLUSIONS
  Three patients with RS were followed up for up to 8 years.
  
  
  CONCLUSIONS
  Our findings suggested that, in contrast to extracutaneous RS, cutaneous RS generally has a less aggressive course with longer survival unless other worse prognostic factors are present.
authors: Department of Pathology, University of Michigan Medical Center, Ann Arbor, Michigan, USA.
authors: Limin
authors: L
authors: Yu
authors: Anshu
authors: A
authors: Bandhlish
authors: Douglas R
authors: DR
authors: Fullen
authors: Lyndon D
authors: LD
authors: Su
authors: Linglei
authors: L
authors: Ma
mesh_terms: D000368:Aged; D002471:Cell Transformation, Neoplastic; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000473:pathology*; D008228:Lymphoma, Non-Hodgkin / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012878:Skin Neoplasms / Q000473:pathology*; D013577:Syndrome; D016159:Tumor Suppressor Protein p53 / Q000378:metabolism
chemical_list: D016159:Tumor Suppressor Protein p53