Meningococcaemia in an adult with hereditary C4b-binding protein deficiency: study of the variations of the protein S system.
Serial studies of the plasma protein C-protein S system were performed during the clinical course of a pregnant woman with meningococcaemia who recovered under therapy. The patient had limited purpura fulminans skin lesions and hereditary C4b-binding protein deficiency was suspected. This diagnosis was confirmed in the patient 1 year after delivery and also by family studies. During the meningococcaemia, an initial mild and transient acquired protein C deficiency was seen but no protein S deficiency was observed despite consumption of the latter protein. As C4b-binding protein partial deficiency is associated with high free protein S and protein S activity, this may have protected against acquired protein S deficiency during meningococcaemia.
Gris
J C
JC
Laboratoire d'Hématologie et Immunologie, CHU, Nîmes, France.
Schved
J F
JF
Martinez
P
P
Bengler
C
C
Lemesle
F
F
Arich
C
C
Sarlat
C
C
D000328:Adult; D002352:Carrier Proteins / Q000378:metabolism*; D002648:Child; D003169:Complement Inactivator Proteins; D005260:Female; D006023:Glycoproteins; D006801:Humans; D008297:Male; D008585:Meningitis, Meningococcal / Q000188:drug therapy / Q000209:etiology*; D011247:Pregnancy; D011251:Pregnancy Complications, Infectious / Q000188:drug therapy / Q000209:etiology; D017293:Protein S / Q000032:analysis*; D011693:Purpura / Q000150:complications
D002352:Carrier Proteins; D003169:Complement Inactivator Proteins; D006023:Glycoproteins; D017293:Protein S