ß-
Conditions
Interventions
All participants will have to stop iron chelating drugs for 4 weeks. After 4 weeks washout period,
the patients will be asked to take L1 (50-
100 mg/kg divided into three daily doses) continually for 12 weeks. L1 (GPO-
ONE®,
Government Pharmaceutical Organization) will be prescribed to every patient without blinding or placebo. There are four visiting time points: before 4-
week washout period,
before starting treatment,
after 4 weeks and after 12 weeks of treatment. In the morning of each visit,
20 ml venous blood sample will be collected from each patient,
and then patients will be given an oral dose of L1 at 17-
33 mg/kg with meals (at 2nd,
3rd and 4th visit only). 13 ml venous blood sample will be obtained from each patient at 2 hours after taking L1. Blood samples will be collected into different tubes,
at t0: 12 ml blood in the tube containing sodium citrate 3.2% for platelet aggregation test
5 ml blood in the serum collection tube for L1 measurement (1 ml),
NTBI determination (1 ml),
oxLDL measurement (2 ml) and P-
selectin ELISA assay (1 ml) and 3 ml blood in the tube containing sodium citrate 3.2% for aPTT/PT test
at t2h: 12 ml blood in the tube containing sodium citrate 3.2% for platelet aggregation test and 1 ml blood in the serum collection tube for L1 measurement.
In polytransfused patients,
blood samples will be obtained before blood transfusion (only containing red blood cells) that is at least 4 weeks following the previous transfusion. Bloods will be collected at Coagulation Laborator
Sponsors
90th Anniversary of Chulalongkorn University Fund
90th Anniversary of Chulalongkorn University Fund
Eligibility
Sex/Gender
All
Age
18 Years to 55 Years
Inclusion criteria
Inclusion criteria:  ß-thalassemia/hemoglobin E major or intermedia patients.  Male and female from 18-55 years old.  No blood transfusion for at least 4 weeks prior to blood collection.  Do not take any medicines or vitamin or dietary supplements known to affect platelet function (except daily folic acid) at least 2 weeks before each assessment of platelet functions.  Appreciate to participate in this study with sign.
Exclusion criteria
Exclusion criteria: - Female who are pregnant or breastfeeding. - Patients with active hepatitis (AST and ALT level are above 3 times the upper limit of normal range), neutropenia, thrombocytopenia, hepatic, renal failure requiring treatment. - ß-thalassemia patients have chronic diseases: hepatic, renal, heart failure; hypertension, diabetes. - Taking antiplatelet or anticoagulant drugs
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| % platelet aggregation every meeting time points, before and 2h after taking drug light transmission aggregation test,sP-selectin level every meeting time points, before and after 2 hours of taking d ELISA | — |
Secondary
| Measure | Time frame |
|---|---|
| Lag time, Slope, delaying time of platelet aggregation every meeting time points, before and 2h after taking drug light transmission aggregation test,deferiprone level every meeting time points, before and 2h after taking drug HPLC,NTBI level every meeting time points, before taking drug colorimetric assay,oxLDL level every meeting time points, before taking drug fluorescent measurement with probe NBD-Pen,PT/aPTT every meeting time points, before taking drug clotting time record,Complete blood count 2nd and 4th visit, before taking drug ,Serum ferritin 2nd and 4th visit, before taking drug | — |
Contacts
Public ContactNgan Tran
Department of Pharmacology and physiology, faculty of Pharmaceutical Sciences, Chulalongkorn Univers
Outcome results
None listed