Cutaneous Richter syndrome: a better place to transform?. (1st February 2015)

• Record Type: Journal Article
• Authors: Kluk, J.; Moonim, M.; Duran, A.; Costa Rosa, J.; Cabeçadas, J.; Alvarez, R.; Cravo, M.; Whittaker, S.; Robson, A.
• Is Part Of: British journal of dermatology. Volume 172:Number 2(2015:Feb.)
• Page Start: 513
• Page End: 521
• Publication Date: 2015-02-01
• Subjects: Dermatology -- Periodicals; Skin -- Diseases -- Periodicals; 616.5
• DOI: 10.1111/bjd.13193
• Languages: English
• ISSNs: 0007-0963

Abstract

Summary: 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.