Article
Proteus syndrome and immunodeficiency
D Hodgea, S A Misbahb, R F Muellerc, E J Glassd, P A J Chetcutia
a Department of
Paediatrics and Child Health, University of Leeds, Leeds, UK, b Department of
Immunology, Leeds General Infirmary, Leeds, UK, c Department of Clinical Genetics, St James's
University Hospital, Leeds, UK, d Department
of Paediatrics, Pinderfields General Hospital, Wakefield, UK
Correspondence to: Dr D Hodge, Academic Unit of Paediatrics and Child Health, University of Leeds, D Floor, Clarendon Wing, The General Infirmary at Leeds, Belmont Grove, Leeds LS2 9NS, UK email: D.Hodge{at}leeds.ac.uk
Accepted 8 November
1999
A 10 year old boy with Proteus syndrome presented with a
pericardial effusion of unknown aetiology. Immunological investigation revealed low serum IgG and IgA, accompanied by low levels of specific antibodies to pneumococcal and haemophilus type B polysaccharides. Circulating lymphocyte surface marker profile revealed T and B cell
lymphopenia. This is the first report of hypogammaglobulinaemia occurring in the Proteus syndrome.
Keywords: Proteus syndrome; hypogammaglobulinaemia; lymphopenia
© 2000 by Archives of Disease in Childhood
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LAKSHMAN, R, FINN, A
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