12 January 2018

Newly-discovered variants of the p53 gene have been linked to the risk and severity of acute lymphoblastic leukaemia (ALL) along with likelihood of developing secondary cancer, researchers have reported.

A team at St. Jude Children's Research Hospital, Memphis, USA identified 22 pathogenic germline variants of the TP53 tumour suppressor gene from samples of 3,858 children with B-cell acute lymphoblastic leukaemia.

Dr Jun Yang and colleagues reported their findings in the Journal of Clinical Oncology.

They explain that knowledge of these variants, which are responsible for about 0.7% of cases, could help guide treatment and follow-up care for certain patients.

The 22 variants were five times more frequent in the leukaemia patients than children without the disease, and were associated with an almost four times increased mortality rate from the disease. They were also linked to a 25% increased risk of developing subsequent cancers, including solid tumours and other leukaemias.

Inherited variants of TP53 are characteristic of Li-Fraumeni syndrome. This hereditary syndrome raises the risk of a variety of cancers, but until recently, had not been associated with leukaemia. This research suggests that the condition may help explain the high rate of secondary cancers seen in childhood ALL survivors with certain TP53 variants.

Dr Yang said: "These germline variations are a double-whammy for carriers. Not only is their risk of developing leukaemia very high, they are also more likely to relapse or develop a second cancer.

"Maybe these patients should avoid certain leukaemia therapies in order to reduce their risk of developing another cancer. I believe this finding may change treatment and follow-up for these high-risk patients."

Source: Qian, M. et al. TP53 Germline Variations Influence the Predisposition and Prognosis of B-Cell Acute Lymphoblastic Leukemia in Children Journal of Clinical Oncology 4 January 2018.

Link: http://ascopubs.org/doi/abs/10.1200/JCO.2017.75.5215


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