title: Primary cutaneous Richter syndrome: prognostic implications and review of the literature.
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.
authors.affiliation: John A. Burns School of Medicine, University of Hawaii at Manoa, Honolulu, Hawaii, USA.
authors.forename: Mika L
authors.initials: ML
authors.lastname: Yamazaki
authors.forename: Christopher A
authors.initials: CA
authors.lastname: Lum
authors.forename: Allan K
authors.initials: AK
authors.lastname: Izumi
mesh_terms: D000368:Aged; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000473:pathology*; D008228:Lymphoma, Non-Hodgkin / Q000473:pathology*; D008297:Male; D011379:Prognosis; D012878:Skin Neoplasms / Q000473:pathology*; D013577:Syndrome