title: How best to manage patients with chronic lymphocytic leuekmia with 17p deletion and/or TP53 mutation?
abstract: Patients with chronic lymphocytic leukemia (CLL) carrying deletion of 17p (17p-) or mutations of TP53 have a uniquely poor prognosis related to increased propensities to progress to symptomatic disease, poor responses to chemo(immuno)therapy and high rates of Richter transformation. Both traditional fludarabine, cyclophosphamide and rituximab (FCR)-based chemoimmunotherapy and alemtuzumab-based regimens are inadequate in controlling 17p- CLL durably, and allogeneic stem cell transplant holds the only prospect for long-term survival. Recent advances in targeted therapies have resulted in novel agents such as B-cell receptor pathway and BCL2 antagonists yielding high response rates in 17p- CLL, but these patients continue to relapse at an increased rate when compared to patients without 17p-. In this review, we discuss the current evidence base for making therapeutic decisions in this difficult disease.
authors: Haematology Department, Peter MacCallum Cancer Center , East Melbourne, Victoria , Australia.
authors: Constantine S
authors: CS
authors: Tam
authors: Stephan
authors: S
authors: Stilgenbauer
mesh_terms: D002872:Chromosome Deletion; D002886:Chromosomes, Human, Pair 17; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000188:drug therapy* / Q000235:genetics*; D009154:Mutation; D058496:Smith-Magenis Syndrome; D016159:Tumor Suppressor Protein p53 / Q000235:genetics*
chemical_list: C495901:TP53 protein, human; D016159:Tumor Suppressor Protein p53
keywords: 17p; Fludarabine; TP53; alemtuzumab; fluorescence in situ hybridization