title: Biological and molecular characterization of a rare case of cutaneous Richter syndrome.
abstract: Richter syndrome (RS) is the transformation of chronic lymphocytic leukemia in a high-grade lymphoma usually presenting nodal and bone marrow involvement. Richter syndrome can be localized at extranodal sites including the gastrointestinal tract, lungs, and skin. Cutaneous RS is an extremely rare disease apparently showing a less aggressive course than common presentations. While nodal RS has been extensively investigated in literature, pathogenesis and prognosis of cutaneous RS are still partially unknown, even if a role of Epstein-Barr virus infection and p53 disruption has been suggested. Herein, we characterized the histopathological, immunohistochemical features and cytogenetics and molecular alterations of a case of cutaneous RS developed after 8 years chronic lymphocytic leukemia history. Moreover, we reviewed the literature reports concerning cutaneous RS and made a focus on biological patterns and prognostic implications.
authors.affiliation: OncoHematology Unit, Fondazione Ca' Granda IRCCS, Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Gianluigi
authors.initials: G
authors.lastname: Reda
authors.affiliation: OncoHematology Unit, Fondazione Ca' Granda IRCCS, Ospedale Maggiore Policlinico, Milan, Italy.|Department of Oncology and Hemato-oncology, University of Milan, Milan, Italy.
authors.forename: Ramona
authors.initials: R
authors.lastname: Cassin
authors.affiliation: OncoHematology Unit, Fondazione Ca' Granda IRCCS, Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Sonia
authors.initials: S
authors.lastname: Fabris
authors.affiliation: Department of Oncology and Hemato-oncology, University of Milan, Milan, Italy.
authors.forename: Gabriella
authors.initials: G
authors.lastname: Ciceri
authors.affiliation: OncoHematology Unit, Fondazione Ca' Granda IRCCS, Ospedale Maggiore Policlinico, Milan, Italy.|Department of Oncology and Hemato-oncology, University of Milan, Milan, Italy.
authors.forename: Bruno
authors.initials: B
authors.lastname: Fattizzo
authors.affiliation: OncoHematology Unit, Fondazione Ca' Granda IRCCS, Ospedale Maggiore Policlinico, Milan, Italy.|Department of Oncology and Hemato-oncology, University of Milan, Milan, Italy.
authors.forename: Mariarita
authors.initials: M
authors.lastname: Sciumè
authors.affiliation: OncoHematology Unit, Fondazione Ca' Granda IRCCS, Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Orofino
authors.affiliation: Hematopathology Service, Division of Pathology, Department of Pathophysiology and Transplantation, IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
authors.forename: Umberto
authors.initials: U
authors.lastname: Gianelli
authors.affiliation: OncoHematology Unit, Fondazione Ca' Granda IRCCS, Ospedale Maggiore Policlinico, Milan, Italy.|Department of Oncology and Hemato-oncology, University of Milan, Milan, Italy.
authors.forename: Antonino
authors.initials: A
authors.lastname: Neri
authors.affiliation: OncoHematology Unit, Fondazione Ca' Granda IRCCS, Ospedale Maggiore Policlinico, Milan, Italy.|Department of Oncology and Hemato-oncology, University of Milan, Milan, Italy.
authors.forename: Agostino
authors.initials: A
authors.lastname: Cortelezzi
mesh_terms: D005260:Female; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000175:diagnosis* / Q000473:pathology; D008875:Middle Aged; D011379:Prognosis; D035583:Rare Diseases; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000473:pathology; D013577:Syndrome
keywords: FISH; chronic lymphocytic leukemia; cutaneous Richter syndrome; immunohistochemistry