title: Richter syndrome first manifesting as cutaneous B-cell lymphoma clonally distinct from primary B-cell chronic lymphocytic leukaemia.
abstract: Richter syndrome (RS) is a transformation to high-grade non-Hodgkin lymphoma in patients with chronic lymphocytic leukaemia (CLL). RS may develop in lymph nodes or rarely extranodally. Skin localization of RS has been described in only a few cases. We present a 77-year-old woman who developed isolated diffuse large B-cell lymphoma (LBCL) in the skin of the nose without any other symptoms of RS. The LBCL in the skin was clonally distinct from the original bone marrow CLL cells. Moreover, LBCL cells were positive for LMP-1 segment of Epstein-Barr virus and overexpressed p53 protein. The patient was successfully treated with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) and adjuvant local radiotherapy.
authors.affiliation: Department of Dermatology, Medical University of Łodz, Łodz, Poland. robaktad@csk.umed.lodz.pl
authors.forename: E
authors.initials: E
authors.lastname: Robak
authors.forename: J
authors.initials: J
authors.lastname: Góra-Tybor
authors.forename: R
authors.initials: R
authors.lastname: Kordek
authors.forename: E
authors.initials: E
authors.lastname: Wawrzyniak
authors.forename: J
authors.initials: J
authors.lastname: Bartkowiak
authors.forename: A
authors.initials: A
authors.lastname: Bednarek
authors.forename: M
authors.initials: M
authors.lastname: Constantinou
authors.forename: B
authors.initials: B
authors.lastname: Kałuzewski
authors.forename: T
authors.initials: T
authors.lastname: Robak
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000473:pathology*; D016393:Lymphoma, B-Cell / Q000188:drug therapy / Q000473:pathology*; D016609:Neoplasms, Second Primary / Q000188:drug therapy / Q000473:pathology; D014411:Neoplastic Stem Cells / Q000473:pathology*; D009669:Nose Neoplasms / Q000188:drug therapy / Q000473:pathology*; D011241:Prednisone / Q000627:therapeutic use; D012878:Skin Neoplasms / Q000188:drug therapy / Q000473:pathology*; D013577:Syndrome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone