Rc. Ratnavel et al., B-cell lymphoma associated with chronic lymphatic leukaemia: two cases with contrasting aggressive and indolent behaviour, BR J DERM, 140(4), 1999, pp. 708-714
The term Richter's syndrome is used to describe the transformation of chron
ic lymphatic leukaemia (CLL) into a high-grade systemic lymphoma and is ass
ociated with a poor prognosis. We have undertaken detailed molecular studie
s in two patients with cutaneous B-cell lymphoma (CBCL) and CLL, Patient 1
exhibited a low-grade CBCL with different immunoglobulin gene rearrangement
s in blood and skin, By contrast, patient 2 showed identical gene rearrange
ments, confirmed by gene sequencing, and died within 4 months of presentati
on, 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 r
earrangement studies with sequencing for the accurate diagnosis of cutaneou
s Richter's syndrome.