WE know that TP53 problems are about the worst you can have with CLL, but we also know that 17p deletion is very rare in newly diagnosed patients. But what about TP53 mutations which seem to be as important?
The Swedish group run by Richard Rosenquist has looked at this.(Leuk Res 2011; 35:272) Among 268 newly diagnosed patients there were 10 who had del 17p at presentation (3.7%). Of these 7 also had TP53 mutations. 8/10 had unmutated VH genes and 5/7 with both TP53 genes affected were Binet stage B at presentation. There were only 3 (1,1%) patients with TP53 mutations and no del 17p; 2 were Binet stage B, but all three had mutated VH genes. The 3 with del 17p without TP53 mutations were all Binet stage A, but 2 had unmutated VH genes.
Even among this group with an early diagnosis and no previous treatment, time to treatment and overall survival was extremely impaired in patients whose TP53 lesion occurred on both chromosomes. Although the numbers were two small to be examined statistically among the 6 patients with only one chromosome involved three did badly. One with del 17p was treated after 5 days and died 55 months later and another with this lesion received treatment after 4 months and died 4 years after diagnosis. The other (with mutated VH genes) is alive and untreated after 9 years. Of the 3 with only mutated TP53, one was treated at 5 months and died 66 months later, while the other two were alive and well at 118 and 121 months although one was treated at 24 months.