Hamblin, Terry J. (2005) Richter's syndrome--the downside of fludarabine? Leukemia Research, 29 (10), 1103-1104. (doi:10.1016/j.leukres.2005.02.020).
Abstract
In 1928 Maurice Richter described an aggressive lymphoma occurring in a patient with CLL [1], and the syndrome that goes by his name is now recognized as a rare but regular culmination of CLL. In the largest series of cases, Robertson et al. [2] reported 39 patients with Richter's syndrome among 1374 with CLL (2.8%). On the other hand, as Thornton et al. [3] report in a recent issue of Leukemia Research, the probably more representative MRC CLL 1, 2 and 3 trials had only 24 out of 1379 patients (1.74%) dying of Richter's syndrome. How accurate are these estimates of prevalence is unclear. Deaths at a young age are investigated more thoroughly than those in old age. In a series of 1011 patients from Rome [4] there were 12 cases of Richter's syndrome among the 207 aged under 55 (5.9%), but only 10 among the 807 cases over 55 (1.2%). Nevertheless, it is hard to escape the conclusion that Richter's syndrome is getting commoner, and in their important study Thornton et al. report [3] an incidence of 12% among 101 patients treated with fludarabine-containing regimens.
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