X-linked lymphoproliferative syndrome (XLP), which is also known as Duncan’s syndrome, is a rare always 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 anaemia. 70% of individuals with XLP die by the age of 10 years without any treatment. The cause of the condition was only found in 1998 so there is still a lot to learn. The best ‘prevention’ is regular top ups of anti-viral medicines, immunoglobulin therapy or steroids – but these are not a cure. Today the only possible cure is a bone marrow transplant – in effect replacing the faulty immune system.

However with advances in genetics a potential cure may now only be a few years away.
 
links for more info on XLP

You could find more informations on the following web sites :

www.xlpresearchtrust.org
 
http://children.webmd.com
 
Dr. Alexandra H. Filipovich continues to play an integral role in identifying and researching XLP as well as various other immunodeficiency’s’. http://www.cincinnatichildrens.org/svc/
find-professional/f/lisa-filipovich.htm
 
 
More about XLP

X-linked Lymphoproliferative Syndrome (XLP) is an extremely rare inherited (primary) immunodeficiency disorder characterized by a defective immune system response to infection with the Epstein-Barr virus (EBV). This herpes virus is common among the general population and causes infectious mononucleosis (IM), usually with no long-lasting effects. However, in individuals with X-Linked Lymphoproliferative Syndrome, exposure to EBV may result in severe, life-threatening infectious mononucleosis; abnormally low levels of antibodies in the blood and body secretions (hypogammaglobulinemia), resulting in increased susceptibility to various infections; malignancies of certain types of lymphoid tissue (B-cell lymphomas); and/or other abnormalities. The range of symptoms and findings associated with XLP may vary from case to case. In addition, the range of effects may change in an affected individual over time. In most cases, individuals with XLP experience an onset of symptoms anytime from approximately six months to 10 years of age.

Approximately half of individuals with X-linked Lymphoproliferative Syndrome experience severe, life-threatening mononucleosis characterized by fever, inflammation and soreness of the throat (pharyngitis), swollen lymph glands, enlargement of the spleen (splenomegaly), enlargement of the liver (hepatomegaly), and/or abnormal functioning of the liver, resulting in yellowing of the skin, mucous membranes, and whites of the eyes (jaundice or icterus). In some cases, individuals who experience life-threatening mononucleosis infection may subsequently have an abnormal increase (i.e., proliferation) of certain white blood cells (lymphocytes and histiocytes) in particular organs, severe liver damage and/or failure, damage to the blood-cell generating bone marrow (hematopoietic marrow cells) that may result in aplastic anemia, and/or other symptoms that may result in life-threatening complications in affected children or adults. Aplastic anemia is characterized by a marked deficiency of all types of blood cells (pancytopenia) including low levels of red blood cells, certain white blood cells, and platelets, specialized red blood cells that function to assist appropriate blood clotting. In individuals with XLP, a decrease in platelets (thrombocytopenia) results in increased susceptibility to bruising and excessive bleeding (hemorrhaging). Because X-linked Lymphoproliferative Syndrome is inherited as an X-linked recessive genetic trait, the disorder is usually fully expressed in males only.