X-linked lymphoproliferative disease (XLP), is one of 100 primary immune deficiency diseases(PIDS). These diseases are disorders in which part of the body’s immune system is missing or does not function properly.
X-linked lymphoproliferative disease (XLP), which is also known as Duncan’s disease, is a rare often fatal disease that affects only boys. To date only about 100 families and 400+ boys have been diagnosed worldwide. It is likely, however, that there are many more cases where the correct diagnosis has not been made.
XLP can have many symptoms including : severe glandular fever, cancer of the blood (lymphoma) and inability to fight off infections and sometimes severe anemia. 70% of boys with XLP die by the age of 10 years, if no treatment has been initiated. The cause of the condition was only found in 1998 so there is still a great deal to learn. Today the only possible cure for XLP is a bone marrow transplant (also known as a stem cell transplantation) – in effect replacing the faulty immune system. However with advances in genetics a potential cure without the need for bone marrow transplantation may be possible in the future.
. www.marrow.org . www.xlpresearchtrust.org
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