Duncan Syndrome

Living reference work entry

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.

Keywords

Hepatitis Lymphoma Anemia Cyclophosphamide Vasculitis 

References

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Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  1. 1.Medical GeneticsShriners Hospitals for ChildrenShreveportUSA
  2. 2.Perinatal and Clinical Genetics, Department of PediatricsLSU Health Sciences CenterShreveportUSA

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