Proteus syndrome and immunodeficiency.
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.
Hodge
D
D
Department of Paediatrics and Child Health, University of Leeds, Leeds, UK. D.Hodge@leeds.ac.uk
Misbah
S A
SA
Mueller
R F
RF
Glass
E J
EJ
Chetcuti
P A
PA
D000361:Agammaglobulinemia / Q000097:blood / Q000150:complications* / Q000276:immunology; D002648:Child; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008231:Lymphopenia / Q000097:blood / Q000150:complications* / Q000276:immunology; D008297:Male; D010490:Pericardial Effusion / Q000209:etiology* / Q000276:immunology; D016715:Proteus Syndrome / Q000097:blood / Q000150:complications* / Q000276:immunology
D007074:Immunoglobulin G