X-Linked Lymphoproliferative Syndrome

Also known as: X-linked lymphoproliferative disease, XLP, Duncan’s syndrome.

What is X-linked lymphoproliferative syndrome?

X-linked lymphoproliferative syndrome is a genetic disease in which the immune system does not work as well as it should. The greatest risk for children with X-linked lymphoproliferative syndrome seems to be from the Epstein-Barr virus, which causes mononucleosis. This virus can cause severe complications for children with X-linked lymphoproliferative syndrome.

What causes X-linked lymphoproliferative syndrome?

X-linked lymphoproliferative syndrome is caused by a genetic mutation. The disease is hereditary and can be passed along from parents to children.

What are the symptoms of X-linked lymphoproliferative syndrome?

Most people have no reaction or develop a mild illness in reaction to the Epstein-Barr virus. In people with X-linked lymphoproliferative syndrome, the immune system overreacts and causes a condition called hemophagocytic lymphohistiocytosis, which causes symptoms such as fever and severe organ damage. People with X-linked lymphoproliferative syndrome also have an increased risk of cancer.

What are X-linked lymphoproliferative syndrome care options?

Many people with X-linked lymphoproliferative syndrome are treated with immunoglobulin replacement therapy to help restore the immune system and reduce the risk of complications from the Epstein-Barr virus. Antibiotics and other medications can also play a role in preventing severe complications from the disease.

Reviewed by: Mislen S Bauer, MD

This page was last updated on: September 09, 2020 11:26 AM