Grants Awarded

Understanding the role of Epstein Barr virus in Hemophagocytic Lymphohistiocytosis

Principal Investigator
Claire Shannon-Lowe
The University of Birmingham
Birmingham, United Kingdom

Date of Award

December 2018

Amount of Award


Layperson Summary

Epstein Barr virus (EBV) is a common virus, infecting up to 95% of the world’s population and persisting within infected individuals for their lifetime. EBV usually infects young children and most infected individuals do not ever know they have been infected because the virus establishes a silent latent infection. EBV usually infects a type of white blood cell called a B lymphocyte. However, in very rare circumstances and for reasons we don’t yet understand, EBV can infect other types of white blood cells called T cells and natural killer (NK) cells. These cell types are not meant to be infected by EBV, so their infection always results in severe diseases including EBVGrants associated hemophagocytic lymphohistiocytosis (EBV-HLH). Unfortunately, the diagnosis of EBV-HLH is difficult and very time consuming and often leads to delays in patients receiving appropriate treatment. Furthermore, even with appropriate treatment, mortality from EBV-HLH is high at 40 – 50%. We, therefore, need to first develop a rapid diagnostic tool to identify which patients have EBV-HLH so these patients can be appropriately and quickly treated. We also need to understand how EBV is causing the disease so that we can develop new strategies to treat the disease and significantly lower the mortality rate. We have developed a new rapid technique to help diagnose EBVHLH and will test this technique in a hospital laboratory to ensure the test is completely accurate and gives no false positive or negatives. This technique can also be used to examine how EBV changes the infected cells and what effect these changes have on each patient. The results so far suggest EBV alters the patient’s immune system by activating immunosuppressive cells. If we can understand how this happens, we can develop new treatment strategies to reverse these effects and efficiently treat the disease.