Abstract
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.
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Chen, H. (2016). Duncan Syndrome. In: Atlas of Genetic Diagnosis and Counseling. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6430-3_71-2
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DOI: https://doi.org/10.1007/978-1-4614-6430-3_71-2
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