Document Detail

Sickle cell disease: relation between procoagulant activity of red blood cells from different phenotypes and in vivo blood coagulation activation.
MedLine Citation:
PMID:  9375737     Owner:  NLM     Status:  MEDLINE    
In the present study we examined if, among other mechanisms, the abnormal exposure of phosphatidylserine at the surface of sickle red blood cells (RBCs) contributes to the hypercoagulability which characterizes homozygous sickle cell disease (SCD). The question was addressed by comparison of the procoagulant properties of RBCs from subjects with various phenotypes (SS, SC and AS) that differ in clinical presentation. As previously reported, SS-RBCs accelerated the prothrombin activation by factor Xa, by decreasing the Km of the reaction compared to normal RBCs. SC-RBCs and AS-RBCs also promoted prothrombin activation although their procoagulant properties were milder compared to SS-RBCs. A significant increase of the thrombin-antithrombin complexes was observed in SS subjects. Prothrombin fragment 1+2 (F1+2) was elevated in half of the SS subjects, but the difference with controls did not reach significance. Elevated levels of thrombin-antithrombin complexes were observed in a number of SC (4/11) and AS (3/12) subjects, but the difference with controls was not significant. A significant correlation was observed between the plasma levels of thrombin-antithrombin complexes in the subjects with SS, AS and AA phenotypes, and the procoagulant properties of RBCs. Our results strongly suggest that the procoagulant properties which characterize SS-RBCs also affect SC-RBCs and AS-RBCs, and that exposure of phosphatidylserine by RBCs contributes to the hypercoagulable state observed in SCD.
D Helley; R Girot; M C Guillin; A Bezeaud
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  British journal of haematology     Volume:  99     ISSN:  0007-1048     ISO Abbreviation:  Br. J. Haematol.     Publication Date:  1997 Nov 
Date Detail:
Created Date:  1997-12-23     Completed Date:  1997-12-23     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0372544     Medline TA:  Br J Haematol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  268-72     Citation Subset:  IM    
Service d'Hématologie et Immunologie, Hôpital Beaujon, Clichy, France.
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MeSH Terms
Anemia, Sickle Cell / blood*
Antithrombin III / metabolism
Blood Coagulation / physiology*
Erythrocytes / metabolism*
Factor Xa / metabolism
Fetal Hemoglobin / metabolism
Middle Aged
Peptide Hydrolases / metabolism
Prothrombin / physiology*
Reg. No./Substance:
0/antithrombin III-protease complex; 9000-94-6/Antithrombin III; 9001-26-7/Prothrombin; 9034-63-3/Fetal Hemoglobin; EC 3.4.-/Peptide Hydrolases; EC Xa

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