Preventing people with immune deficiencies from developing cancer

Background

One of the main jobs of our immune system is to protect us from infectious diseases. However, some people have errors in their DNA that can cause immune deficiencies. For these people, infectious diseases that would usually only cause minor symptoms in most people can proliferate throughout the body causing significant symptoms, tissue damage and, in some cases, can lead to cancer. This means people with deficient immune systems are significantly more likely to develop cancer than those with healthy immune systems.

Professor Stuart Tangye has been leading pioneering research on the link between the immune system and cancer with funding from Cancer Council NSW since 2002. Professor Tangye and his team discovered a link between a particular immune deficiency, X-linked lymphoproliferative disease (XLP), the Epstein-Barr virus and the development of cancer.

The Epstein-Barr virus is very common, with around 90% of people in the world having it at some point in their lives – sometimes without even realising. It can cause cold or flu-like symptoms, or no symptoms at all. However, people with XLP cannot control this virus. As a result, the virus runs rampant in their bodies, causing tissue damage and can often lead to non-Hodgkin’s lymphoma. The Epstein-Barr virus has also been linked with six other types of cancer.

The research

In this project, the team has been investigating if it’s possible to boost the immune system of people with XLP to build their defences against the Epstein-Barr virus and prevent them from developing cancer. By analysing the immune responses of patients with XLP, Professor Tangye has been homing in on exactly why their immune system fails to fight the Epstein-Barr virus.

They found the two types of immune cells which are primarily responsible for controlling viruses are unable to recognise the Epstein-Barr virus and activate the immune system to attack. With this stage of the immune response pinpointed, the team has been investigating how to boost the immune system of people with XLP, build their defences against the Epstein-Barr virus and potentially prevent them getting cancer.