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Proteus syndrome and immunodeficiency
  1. D Hodgea,
  2. S A Misbahb,
  3. R F Muellerc,
  4. E J Glassd,
  5. P A J Chetcutia
  1. aDepartment of Paediatrics and Child Health, University of Leeds, Leeds, UK, bDepartment of Immunology, Leeds General Infirmary, Leeds, UK, cDepartment of Clinical Genetics, St James's University Hospital, Leeds, UK, dDepartment of Paediatrics, Pinderfields General Hospital, Wakefield, UK
  1. 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

Abstract

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.

  • Proteus syndrome
  • hypogammaglobulinaemia
  • lymphopenia

https://doi.org/10.1136/adc.82.3.234

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