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Cutaneous Richter syndrome: a better place to transform?
Author(s) -
Kluk J.,
Moonim M.,
Duran A.,
Costa Rosa J.,
Cabeçadas J.,
Alvarez R.,
Cravo M.,
Whittaker S.,
Robson A.
Publication year - 2015
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/bjd.13193
Subject(s) - medicine , psychoanalysis , dermatology , psychology
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.