In 1975, Purtilo et al. (1975) reported the Duncan pedigree in which 6 of 18 males died of a lymphoproliferative disorder. The boys, aged between 2 and 19 years, exhibited a progressive combined variable immunodeficiency disease characterized by benign or malignant proliferation of lymphocytes, histiocytosis, and alterations in concentrations of serum immunoglobulins. This condition is now known as Duncan syndrome or X-linked lymphoproliferative syndrome (XLP) which is a rare T and NK cell immune deficiency which most frequently presents as fulminant infectious mononucleosis following infection with the Epstein-Barr virus (EBV) in most affected boys, leading to death in 50 % of the cases.
KeywordsHepatitis Lymphoma Anemia Cyclophosphamide Vasculitis
- Filipovich, A., Johnson, J., Zhang, K., et al. (2013). Lymphoproliferative disease, X-linked [Duncan disease, XLPD]. Gene Review. Retrieved 19 Sept 2013. Available at: http://www.ncbi.nlm.nih.gov/books/NBK1406/
- Seiter, K. (2015). X-linked lymphoproliferative syndrome. eMedicine from WebMD. Updated 26 Nov 2015. Available at: http://emedicine.medscape.com/article/203780-overview