FcgammaRIIIb and complement component C7 codeficiency in a patient with recurrence of fulminant meningococcal septic shock.
Individuals with deficiencies of the late components of complement exhibit a susceptibility to the recurrence of meningococcal disease with a usually mild clinical presentation. We report the recurrence of fulminant meningococcal disease in a complement component C7-deficient patient. We found a total deficiency of FcgammaRIIIb on neutrophils, which could partially explain the unusually severe clinical presentation.
Debard
Anne-Lise
AL
Immunology Laboratory, Lyon-Sud University Hospital, Pierre-Benite, France.
Lamy
Brigitte
B
Monneret
Guillaume
G
Mira
Jean-Paul
JP
Pachot
Alexandre
A
Kleijer
Marion
M
Aillaud
Marie-Françoise
MF
Boibieux
André
A
Bienvenu
Jacques
J
Carret
Gérard
G
Fournier
Gérard
G
Bohé
Julien
J
D000293:Adolescent; D015703:Antigens, CD / Q000378:metabolism; D016470:Bacteremia; D003184:Complement C7 / Q000172:deficiency* / Q000235:genetics; D005260:Female; D058851:GPI-Linked Proteins; D015870:Gene Expression; D020022:Genetic Predisposition to Disease; D005838:Genotype; D006801:Humans; D008589:Meningococcal Infections / Q000235:genetics / Q000378:metabolism*; D009504:Neutrophils / Q000378:metabolism; D017452:Receptors, IgG / Q000172:deficiency* / Q000378:metabolism; D012008:Recurrence; D012772:Shock, Septic / Q000235:genetics / Q000378:metabolism*
D015703:Antigens, CD; D003184:Complement C7; C428200:FCGR3B protein, human; D058851:GPI-Linked Proteins; D017452:Receptors, IgG