Primary cutaneous Richter syndrome: Prognostic implications and review of the literature - 08/08/11
Reprint requests: Allan K. Izumi, MD, 1380 Lusitana St, Ste 412, Honolulu, HI 96813.Abstract |
The term Richter syndrome (RS) describes the transformation of chronic lymphocytic leukemia into a high-grade lymphoma. RS occurs in 3% to 10% of chronic lymphocytic leukemia cases, and its onset is often characterized by the abrupt development of systemic symptoms (eg, fever in the absence of infection, night sweats, and weight loss), progressive lymphadenopathy, and hepatosplenomegaly. RS frequently arises in the lymph nodes or bone marrow, and rarely presents with extranodal involvement, which includes the gastrointestinal tract, eye, testis, central nervous system, lung, kidney, and skin. We review the literature regarding the clinical course and treatment of RS, present a patient with primary cutaneous RS, and discuss the prognostic implications.
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Vol 60 - N° 1
P. 157-161 - janvier 2009 Retour au numéro
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