Changes of hemostatic factors in patients with hemoglobinopathies

In this study, protein C (PC), protein S (PS), heparin cofactor II (HCFII), prothrombin fragment 1+2(PF1,2), thrombin-antithrombin III complex (TAT), von Willebrand factor (vWF) and thrombomodulin (TM) were investigated in 13 patients with beta thalassemia intermedia (TI) not requiring transf...

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Main Authors: A F Oner, A Gürgey, H Okur, S Kirazli, F Gümrük, C Altay
Format: Article
Language:English
Published: Hacettepe University Institute of Child Health 1999-07-01
Series:The Turkish Journal of Pediatrics
Online Access:https://turkjpediatr.org/article/view/3239
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author A F Oner
A Gürgey
H Okur
S Kirazli
F Gümrük
C Altay
author_facet A F Oner
A Gürgey
H Okur
S Kirazli
F Gümrük
C Altay
author_sort A F Oner
collection DOAJ
description In this study, protein C (PC), protein S (PS), heparin cofactor II (HCFII), prothrombin fragment 1+2(PF1,2), thrombin-antithrombin III complex (TAT), von Willebrand factor (vWF) and thrombomodulin (TM) were investigated in 13 patients with beta thalassemia intermedia (TI) not requiring transfusion, six patients with sickle cell disease (SCD), and seven patients with HbS-beta thalassemia (S-BT) who were not in crisis. These hemostatic parameters were also studied in 12 healthy children assigned as a control group. Protein C and Protein S (PC-PS) were found to be decreased in TI patients and normal in S-BT patients. PC was decreased in SCD patients. In the patients with TI and SCD, the mean PF1,2 level was elevated, whereas the TAT level was not statistically different from that of the control group. These results suggested that in patients with hemoglobinopathies: a) decreased natural anticoagulants and b) enhanced procoagulant activation have been encountered. Other unexpected and interesting results of this study are the decreased vWF and elevated HCFII levels in all three patient groups.
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issn 0041-4301
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publishDate 1999-07-01
publisher Hacettepe University Institute of Child Health
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series The Turkish Journal of Pediatrics
spelling doaj-art-c3b0ea4b8b4c4af8af533cb0886ff8422025-08-20T02:58:14ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64211999-07-01413Changes of hemostatic factors in patients with hemoglobinopathiesA F Oner0A GürgeyH OkurS KirazliF GümrükC AltayDepartment of Pediatrics, Hacettepe University Faculty of Medicine, Ankara. In this study, protein C (PC), protein S (PS), heparin cofactor II (HCFII), prothrombin fragment 1+2(PF1,2), thrombin-antithrombin III complex (TAT), von Willebrand factor (vWF) and thrombomodulin (TM) were investigated in 13 patients with beta thalassemia intermedia (TI) not requiring transfusion, six patients with sickle cell disease (SCD), and seven patients with HbS-beta thalassemia (S-BT) who were not in crisis. These hemostatic parameters were also studied in 12 healthy children assigned as a control group. Protein C and Protein S (PC-PS) were found to be decreased in TI patients and normal in S-BT patients. PC was decreased in SCD patients. In the patients with TI and SCD, the mean PF1,2 level was elevated, whereas the TAT level was not statistically different from that of the control group. These results suggested that in patients with hemoglobinopathies: a) decreased natural anticoagulants and b) enhanced procoagulant activation have been encountered. Other unexpected and interesting results of this study are the decreased vWF and elevated HCFII levels in all three patient groups. https://turkjpediatr.org/article/view/3239
spellingShingle A F Oner
A Gürgey
H Okur
S Kirazli
F Gümrük
C Altay
Changes of hemostatic factors in patients with hemoglobinopathies
The Turkish Journal of Pediatrics
title Changes of hemostatic factors in patients with hemoglobinopathies
title_full Changes of hemostatic factors in patients with hemoglobinopathies
title_fullStr Changes of hemostatic factors in patients with hemoglobinopathies
title_full_unstemmed Changes of hemostatic factors in patients with hemoglobinopathies
title_short Changes of hemostatic factors in patients with hemoglobinopathies
title_sort changes of hemostatic factors in patients with hemoglobinopathies
url https://turkjpediatr.org/article/view/3239
work_keys_str_mv AT afoner changesofhemostaticfactorsinpatientswithhemoglobinopathies
AT agurgey changesofhemostaticfactorsinpatientswithhemoglobinopathies
AT hokur changesofhemostaticfactorsinpatientswithhemoglobinopathies
AT skirazli changesofhemostaticfactorsinpatientswithhemoglobinopathies
AT fgumruk changesofhemostaticfactorsinpatientswithhemoglobinopathies
AT caltay changesofhemostaticfactorsinpatientswithhemoglobinopathies