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.

Biological and molecular characterization of a rare case of cutaneous Richter syndrome / G. Reda, R. Cassin, S. Fabris, G. Ciceri, B. Fattizzo, M. Sciumè, N. Orofino, U. Gianelli, A. Neri, A. Cortelezzi. - In: HEMATOLOGICAL ONCOLOGY. - ISSN 0278-0232. - 35:4(2017 Dec), pp. 869-874. [10.1002/hon.2327]

Biological and molecular characterization of a rare case of cutaneous Richter syndrome

G. Reda
Primo
;
R. Cassin
Secondo
;
S. Fabris;G. Ciceri;B. Fattizzo;M. Sciumè;N. Orofino;U. Gianelli;A. Neri
Penultimo
;
A. Cortelezzi
Ultimo
2017-12

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.
FISH; chronic lymphocytic leukemia; cutaneous Richter syndrome; immunohistochemistry
Settore MED/15 - Malattie del Sangue
12-lug-2016
Article (author)
File in questo prodotto:
File Dimensione Formato  
Reda_et_al-2016-Hematological_Oncology.pdf

accesso riservato

Tipologia: Publisher's version/PDF
Dimensione 239.18 kB
Formato Adobe PDF
239.18 kB Adobe PDF   Visualizza/Apri   Richiedi una copia
Pubblicazioni consigliate

Caricamento pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/422168
Citazioni
  • ???jsp.display-item.citation.pmc??? 0
  • Scopus 2
  • ???jsp.display-item.citation.isi??? 1
social impact