title: Protein C and protein S deficiency in thalassemic patients.
abstract: To investigate the status of the protein C-protein S anticoagulant pathway in thalassemic patients, we measured protein C and protein S levels of plasma of 30 adults and 18 children with beta-thalassemia/HbE disease, beta-thalassemia major and HbE disease. Mean +/- 1 SD values of protein C, protein S and other coagulant proteins produced by the liver were as follows: protein C 50.4 +/- 17.2%; protein S 58.8 +/- 25.5%; antithrombin III 78.1 +/- 12.8%; PLG 86.4 +/- 18.4%; prothrombin 71.0 +/- 13.1%; factor VII 72.7 +/- 21.5%; and factor X 79.2 +/- 15.6%. Protein C and protein S levels of thalassemic patients were significantly lower than those of other coagulant proteins produced by the liver. Decrease in protein C level was stronger than that of proteins S. gamma-Carboxylated protein C levels of splenectomized patients were significantly lower than those of nonsplenectomized patients. Severe decrease of protein C and protein S may be responsible for occurrence of thrombosis in thalassemic patients.
authors.affiliation: Department of Pediatrics, University of Occupational and Environmental Health Japan, Kitakyushu.
authors.forename: A
authors.initials: A
authors.lastname: Shirahata
authors.forename: Y
authors.initials: Y
authors.lastname: Funahara
authors.forename: N
authors.initials: N
authors.lastname: Opartkiattikul
authors.forename: S
authors.initials: S
authors.lastname: Fucharoen
authors.forename: V
authors.initials: V
authors.lastname: Laosombat
authors.forename: K
authors.initials: K
authors.lastname: Yamada
mesh_terms: D000293:Adolescent; D000328:Adult; D000410:Alanine Transaminase; D001779:Blood Coagulation Factors / Q000737:chemistry; D002648:Child; D002675:Child, Preschool; D006446:Hemoglobin E; D006453:Hemoglobinopathies / Q000097:blood* / Q000150:complications / Q000601:surgery; D006785:Hospitals, University; D006801:Humans; D007223:Infant; D008111:Liver Function Tests; D008875:Middle Aged; D011486:Protein C / Q000737:chemistry*; D020151:Protein C Deficiency; D017293:Protein S / Q000097:blood*; D018455:Protein S Deficiency; D012307:Risk Factors; D012709:Serum Albumin / Q000032:analysis; D013156:Splenectomy; D013785:Thailand / Q000453:epidemiology; D013923:Thromboembolism / Q000097:blood / Q000453:epidemiology / Q000209:etiology; D017086:beta-Thalassemia / Q000097:blood* / Q000150:complications / Q000601:surgery
chemical_list: D001779:Blood Coagulation Factors; D011486:Protein C; D017293:Protein S; D012709:Serum Albumin; D006446:Hemoglobin E; D000410:Alanine Transaminase