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Abnormally high level of C4b binding protein and deficiency of free fraction of protein S in a patient with systemic lupus erythematosus and recurrent thromboses.

Abstract
A patient with systemic lupus erythematosus and numerous thrombotic accidents was found to have a deficiency of the free fraction of protein S, most likely due to an abnormally high level of C4b binding protein. These abnormalities, unique in comparison to 4 other patients with hypercoagulation states, were partially corrected with cyclophosphamide. There was no evidence of a consumption of complement components. The interactions between the hemostasis system and the complement cascade are discussed.
AuthorsA J Stankiewicz, M Steiner, E V Lally, S R Kaplan
JournalThe Journal of rheumatology (J Rheumatol) Vol. 18 Issue 1 Pg. 82-7 (Jan 1991) ISSN: 0315-162X [Print] Canada
PMID1827159 (Publication Type: Case Reports, Journal Article, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Blood Proteins
  • Carrier Proteins
  • Complement Inactivator Proteins
  • Glycoproteins
  • Protein S
  • Complement C4b
  • Cyclophosphamide
Topics
  • Adult
  • Blood Proteins (deficiency)
  • Carrier Proteins (analysis)
  • Complement C4b (metabolism)
  • Complement Inactivator Proteins
  • Cyclophosphamide (therapeutic use)
  • Female
  • Glycoproteins (deficiency)
  • Humans
  • Immunoelectrophoresis, Two-Dimensional
  • Lupus Erythematosus, Systemic (blood, complications)
  • Protein S
  • Recurrence
  • Thrombosis (blood, complications)

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