Protein C levels in beta-thalassemia major patients in the east Nile delta of Egypt.
OBJECTIVE
Thalassemic patients have an increased risk for thromboembolic complications. To determine if this might be due to a deficiency in protein C, we investigated the status of the protein C anticoagulant pathway in thalassemia major patients and its relationship to the hypercoagulable state.


METHODS
Fifty patients with beta-thalassemia major (30 non-splenectomized and 20 splenectomized) and 20 healthy children as a control group were tested for levels of serum ferritin, liver enzymes, serum albumin, fibrinogen, protein C and protein S, thrombin antithrombin complex (TAT) and D-dimer.


RESULTS
Thalassemic patients had lower levels of protein C and S and higher levels of D-dimer and TAT than the control group. These findings were more obvious in splenectomized patients and in those with infrequent blood transfusion.


CONCLUSIONS
Protein C plays a major role in the hypercoagulable state in thalassemic patients. These findings raise the issue as to whether it would be cost-beneficial to recommend prophylactic antithrombotic therapy in high-risk thalassemic patients. A wider prospective study is necessary to delineate under which circumstances therapy might be needed, and at what level of protein C deficiency to start prophylactic antithrombotic therapy.
Hassan
Tamer H
TH
Pediatric and Clinical Patholgoy Department, Zagazig University, Zagazig, Egypt. dr.tamerhassan@yahoo.com
Elbehedy
Rabab M
RM
Youssef
Doaa M
DM
Amr
Ghada E
GE
D000990:Antithrombin III; D002648:Child; D004534:Egypt; D005260:Female; D005293:Ferritins / Q000097:blood; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis / Q000378:metabolism; D006801:Humans; D008297:Male; D010447:Peptide Hydrolases / Q000097:blood; D011486:Protein C / Q000032:analysis* / Q000378:metabolism; D017293:Protein S / Q000032:analysis / Q000378:metabolism; D012307:Risk Factors; D012709:Serum Albumin / Q000032:analysis / Q000378:metabolism; D013156:Splenectomy; D013923:Thromboembolism / Q000097:blood / Q000209:etiology / Q000517:prevention & control; D017086:beta-Thalassemia / Q000097:blood* / Q000150:complications / Q000601:surgery
D005338:Fibrin Fibrinogen Degradation Products; D011486:Protein C; D017293:Protein S; D012709:Serum Albumin; C046193:antithrombin III-protease complex; C036309:fibrin fragment D; D000990:Antithrombin III; D005293:Ferritins; D010447:Peptide Hydrolases