Meningococcaemia in an adult with hereditary C4b-binding protein deficiency: study of the variations of the protein S system

Blood Coagul Fibrinolysis. 1992 Oct;3(5):673-7. doi: 10.1097/00001721-199210000-00027.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Carrier Proteins / metabolism*
  • Child
  • Complement Inactivator Proteins*
  • Female
  • Glycoproteins*
  • Humans
  • Male
  • Meningitis, Meningococcal / drug therapy
  • Meningitis, Meningococcal / etiology*
  • Pregnancy
  • Pregnancy Complications, Infectious / drug therapy
  • Pregnancy Complications, Infectious / etiology
  • Protein S / analysis*
  • Purpura / complications

Substances

  • Carrier Proteins
  • Complement Inactivator Proteins
  • Glycoproteins
  • Protein S