title: Cutaneous Richter syndrome: a better place to transform?
abstract: The development of high-grade lymphoma in patients with chronic lymphocytic leukaemia is known as Richter syndrome (RS) and is associated with a grave prognosis, with a mean survival of 8 months despite treatment. Cutaneous RS has been described in a handful of cases and may be associated with a better outcome than the more common extracutaneous variants. We review the literature with particular emphasis on pathogenesis, treatment and survival of RS. We postulate that the absence of B symptoms and a normal lactate dehydrogenase level, presumably reflecting localized or limited disease, and a lower tumour burden, may explain the apparently better survival in some patients with cutaneous RS than with extracutaneous variants.
authors.affiliation: Skin Tumour Unit, 2nd Floor, Block C, South Wing, St. John's Institute of Dermatology, St Thomas' Hospital, Westminster Bridge Road, London, SE1 7EH, U.K.
authors.forename: J
authors.initials: J
authors.lastname: Kluk
authors.forename: M
authors.initials: M
authors.lastname: Moonim
authors.forename: A
authors.initials: A
authors.lastname: Duran
authors.forename: J
authors.initials: J
authors.lastname: Costa Rosa
authors.forename: J
authors.initials: J
authors.lastname: Cabeçadas
authors.forename: R
authors.initials: R
authors.lastname: Alvarez
authors.forename: M
authors.initials: M
authors.lastname: Cravo
authors.forename: S
authors.initials: S
authors.lastname: Whittaker
authors.forename: A
authors.initials: A
authors.lastname: Robson
mesh_terms: D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000188:drug therapy*; D008297:Male; D011241:Prednisone / Q000008:administration & dosage; D000069283:Rituximab; D012878:Skin Neoplasms / Q000188:drug therapy*; D013577:Syndrome; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; C571759:R-CHOP protocol; D000069283:Rituximab; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone