The Change of Coagulation Markers in Children With β-thalassemia Disease After Stem Cell Transplantation

Mahidol University

Status: Completed

Conditions: Thalassemia

Study type: Observational

Funder types: Other

Identifiers

NCT00789516

ID11-48-16

Details and patient eligibility

About

Hypercoagulable state is well recognized in patients with β-thalassemia. Evidences of hypercoagulability include abnormal expression of phosphatidylserine on red blood cell (rbc) surface and consequent increased platelet activation and thrombin generation. In addition, a reduction of anticoagulants i.e. proteins C and S and antithrombin (AT) was demonstrated. However, coagulable state in patients with β-thalassemia following stem cell transplantation (SCT) has not been characterized.

Full description

Hypercoagulable state is well recognized in patients with β-thalassemia. Evidences of hypercoagulability include abnormal expression of phosphatidylserine on red blood cell (rbc) surface and consequent increased platelet activation and thrombin generation. In addition, a reduction of anticoagulants i.e. proteins C and S and antithrombin (AT) was demonstrated. However, coagulable state in patients with β-thalassemia following stem cell transplantation (SCT) has not been characterized.Therefore, the objective is to compare coagulation markers and anticoagulants among β-thalassemics with and without SCT and normal control (NC).The subjects will be classified into 3 groups; β-thalassemia post SCT (Thal-SCT), β-thalassemia treated with regular transfusion (Thal-RT) and NC. Blood samples will be tested for annexin V (an index of abnormal expression of phosphatidylserine on rbc surface), markers of activation of coagulation system (thrombin antithrombin complex (TAT), prothrombin fragment (F1+2), and D-dimer) and anticoagulants (proteins C and S and AT).

Enrollment: 60 patients

Sex: All

Ages: 1 to 18 years old

Volunteers: Accepts Healthy Volunteers

Inclusion criteria

Group 1: beta thalassemia major or beta thalassemia / Hb E who receive regular transfusion therapy (Thal- RT). The baseline Hct was more than 24% for at least 6 months.

Group 2: beta thalassemia major or beta thalassemia / Hb E post SCT (Thal-SCT) who were discontinued immunosuppressive drugs.

Group 3: Normal children (NC) who had normal Hb/Hct and MCV for age

Exclusion criteria

Children with beta thalassemia major or beta thalassemia / Hb E who have co-diseases such as immune hemolytic anemia, infection, or inflammatory diseases

Trial design

60 participants in 3 patient groups

1

Description:

Normal control

2

Description:

B thalassemia regular transfusion

3

Description:

B thalassemia post transplantation