Treatment of B-cell Prolymphocytic Leukemia with TP53 Deletion or Mutation
Clinical scenario
This protocol applies to patients with B-cell prolymphocytic leukemia (B-PLL) who carry a TP53 deletion and/or mutation — a molecular finding that defines a distinct treatment population.
TP53 deletion / mutation
The presence of TP53 deletion or mutation in B-PLL is a clinically important distinction. Patients with nonfunctional TP53 are not candidates for the combination chemo-immunotherapy approach used in TP53-intact disease; a separate treatment strategy applies to this subgroup.
Treatment approach (partial)
In this setting, management may involve a monoclonal antibody or a B-cell receptor (BCR) inhibitor — the complete structured regimen, including specific agent selection, is available in the full protocol.
References
DOI: 10.1182/asheducation-2015.1.361
- For T-PLL, first-line therapy is with intravenous alemtuzumab and for B-PLL is with combination chemo-immunotherapy for patients with normal TP53 and with alemtuzumab or BCR inhibitors for those with deletions or mutations of TP53.
- For those patients with nonfunctional TP53, alemtuzumab has historically been the mainstay of treatment and, although no longer licensed in CLL, is available via a patient access scheme.
- In the absence of any prospective clinical trial data for B-PLL, it would seem that BCR inhibitors may be an effective therapeutic option, especially in 17p-deleted cases, and experience with these and other novel agents is likely to emerge over the next few years.