B-cell lymphoma associated with chronic lymphatic leukaemia: two cases with contrasting aggressive and indolent behaviour.
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.
Ratnavel
R C
RC
Department of Dermatopathology, St John's Institute of Dermatology, United Medical and Dental Schools (UMDS), UMDS, London SE1 7EH, U.K. ratnavel@epulse.net
Dunn-Walters
D K
DK
Boursier
L
L
Frazer-Andrews
E
E
Orchard
G
G
Russell-Jones
R
R
Calonje
E
E
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