title: B-cell lymphoma associated with chronic lymphatic leukaemia: two cases with contrasting aggressive and indolent behaviour.
abstract: The term Richter's syndrome is used to describe the transformation of chronic lymphatic leukaemia (CLL) into a high-grade systemic lymphoma and is associated with a poor prognosis. We have undertaken detailed molecular studies in two patients with cutaneous B-cell lymphoma (CBCL) and CLL. Patient 1 exhibited a low-grade CBCL with different immunoglobulin gene rearrangements in blood and skin. By contrast, patient 2 showed identical gene rearrangements, confirmed by gene sequencing, and died within 4 months of presentation. The latter patient fulfilled the criteria for a diagnosis of cutaneous Richter's syndrome, whereas the former patient demonstrated the coincidence of CLL with a primary CBCL. Our results highlight the importance of gene rearrangement studies with sequencing for the accurate diagnosis of cutaneous Richter's syndrome.
authors.affiliation: Department of Dermatopathology, St John's Institute of Dermatology, United Medical and Dental Schools (UMDS), UMDS, London SE1 7EH, U.K. ratnavel@epulse.net
authors.forename: R C
authors.initials: RC
authors.lastname: Ratnavel
authors.forename: D K
authors.initials: DK
authors.lastname: Dunn-Walters
authors.forename: L
authors.initials: L
authors.lastname: Boursier
authors.forename: E
authors.initials: E
authors.lastname: Frazer-Andrews
authors.forename: G
authors.initials: G
authors.lastname: Orchard
authors.forename: R
authors.initials: R
authors.lastname: Russell-Jones
authors.forename: E
authors.initials: E
authors.lastname: Calonje
mesh_terms: D000368:Aged; D005260:Female; D015321:Gene Rearrangement / Q000502:physiology; D005803:Genes, Immunoglobulin / Q000502:physiology; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000276:immunology / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008297:Male; D012878:Skin Neoplasms / Q000150:complications* / Q000276:immunology / Q000473:pathology