S. Jelic et al., RICHTER SYNDROME WITH EMPHASIS ON LARGE-CELL NON-HODGKIN-LYMPHOMA IN PREVIOUSLY UNRECOGNIZED SUBCLINICAL CHRONIC LYMPHOCYTIC-LEUKEMIA, Neoplasma, 44(1), 1997, pp. 63-68
The authors report seven cases of Richter's syndrome, i.e. of large-ce
ll non-Hodgkin's lymphoma (NHL) arising in association with chronic ly
mphocytic leukemia (CLL). Six patients had the recently recognized var
iant of this syndrome, occurring in patients with previously undiagnos
ed subclinical CLL. All patients were treated with aggressive chemothe
rapy and a complete response of large cell NHL was achieved in 4/7. A
complete response of NHL was observed in 3 out of 6, and a partial res
ponse in 2/6 patients with simultaneous occurrence of subclinical CLL
and large cell NHL (response rate 5/6). Our findings might suggest tha
t patients with Richter syndrome occurring in previously undiagnosed s
ubclinical CLL could represent a better prognostic group in the overal
l population of patients with large cell NHL transformation of CLL.