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.
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.
Stankiewicz
A J
AJ
Division of Hematology/Oncology, Memorial Hospital of Rhode Island, Pawtucket 02860.
Steiner
M
M
Lally
E V
EV
Kaplan
S R
SR
D000328:Adult; D001798:Blood Proteins / Q000172:deficiency; D002352:Carrier Proteins / Q000032:analysis*; D015935:Complement C4b / Q000378:metabolism; D003169:Complement Inactivator Proteins; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006023:Glycoproteins / Q000172:deficiency*; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional; D008180:Lupus Erythematosus, Systemic / Q000097:blood* / Q000150:complications; D017293:Protein S; D012008:Recurrence; D013927:Thrombosis / Q000097:blood / Q000150:complications*
D001798:Blood Proteins; D002352:Carrier Proteins; D003169:Complement Inactivator Proteins; D006023:Glycoproteins; D017293:Protein S; D015935:Complement C4b; D003520:Cyclophosphamide