Richter's transformation, or Richter's syndrome, is an uncommon clinicopathological condition observed in about 5% to 10% of patients with chronic lymphocytic leukemia (CLL). This review summarizes advances in our understanding of the pathobiology and in the management of Richter's transformation in patients with CLL.
One strategy would be to consider “early treatment” for patients with deletion 17p, with the goal being to delay disease progression. This strategy is currently being explored in several prospective clinical trials employing treatment regimens such as FCR, lenalidomide (Revlimid), alemtuzumab (Campath), ofatumumab (Arzerra), and others.
ONCOLOGY talks with Dr. Susan O’Brien, professor in the department of leukemia at the MD Anderson Cancer Center. Dr. O’Brien will be one of the presenters at the upcoming ASCO session on therapies for chronic lymphocytic leukemia, and she gives us a preview of what some of the highlights of the session are likely to be, as well as some insights into her own work.
Chronic lymphocytic leukemia (CLL) is the most common adult leukemia in the Western
hemisphere, accounting for 30% of the leukemias in this population. The disease results from a
clonal expansion of small B-lymphocytes. CLL always involves the bone marrow and peripheral
blood. The disease also can be demonstrated in lymph nodes, liver, and spleen.
Chronic lymphocytic leukemia (CLL) is a clonal malignancy that results from
expansion of the mature lymphocyte compartment. This expansion is a
consequence of prolonged cell survival, despite a low proliferative index. The
affected lymphocytes are of B-cell lineage in 95% of cases, and the remaining
cases involve T lymphocytes, likely representing a distinct disorder.
Dr. Nabhan and his coauthors
have written a comprehensive
review of the use of monoclonal
antibodies in the treatment of
chronic lymphocytic leukemia (CLL).
They have highlighted important
clinical trials with newer antibodies,
including apolizumab (Hu1D10,
Remitogen) and IDEC-152 (anti-
CD23). The authors concisely describe
the use of rituximab (Rituxan)
and alemtuzumab (Campath) as single
agents and in combination therapy.
Both antibodies have efficacy in
the treatment of CLL, but both have
limitations when used as single
Wild-type bcl-2 protein is normally found within the bilaminar membrane of the mitochondrion, where it is believed to negatively regulate the release of cytochrome C into the cytoplasm after an apoptotic signal has triggered dimerization of bax protein.