Rare blood disease XLP
By Steve Benowitz
(Left-right): Jennifer Cannons, Roseanne Zhao (lead author), Pam Schwartzberg
Special to NHGRI
A rare, genetic disease found only in boys is helping researchers at the National Human Genome Research Institute (NHGRI) unlock secrets about how the body fends off infection. Studying a mouse model of an inherited disease called X-linked lymphoproliferative syndrome (XLP), they have discovered new details explaining how a missing protein can disrupt communication between two important types of white blood cells, T and B cells, which play key roles in immunity.
The findings also help explain why in some patients with XLP, miscommunication between T and B cells can be deadly, turning it into an often fatal immune disorder triggered by infection with an otherwise non-lethal, common virus, Epstein-Barr virus (EBV). The findings, Positive and Negative Signaling through SLAM Receptors Regulate Synapse Organization and Thresholds of Cytolysis, were reported in the June 7 online version of the journal Immunity.
XLP affects about one in a million boys. About half of these children experience a severe immune response to infection with EBV, resulting in symptoms that can include fever, hepatitis, an enlarged spleen, abnormally low numbers of antibodies, and, in some cases, lymphoma and other blood disorders. EBV infects B cells, and normally, the body clears such infections, but individuals with XLP are unable to kill these EBV-infected cells.
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