Duncan's syndrome

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Duncan's syndrome is a rare X-linked recessive lymphoproliferative disorder in which infection by Epstein Barr virus may result in an acutely lethal mononucleosis, development of autoimmune disease, or the delayed appearance of immunoblastic or non-Hodgkin's lymphoma.

It is due to defective T-cell function resulting in failure to recognise and destroy EBV-infected B cells. Affected males may be of any age from 6 months to over 20 years.

The condition may explain the link between EBV infection and Burkitt's lymphoma, the latter resulting when a defective cell mediated response is combined with an 8:14 autosomal translocation.

Last reviewed 01/2018