An Open Label Study to Evaluate the Pharmacokinetics, Safety, Tolerability and Efficacy of Deferasirox Administered to Chinese Patients With β-thalassemia Major Aged From 2 to Less Than 6 Years Old (MACS2163)
β-thalassemia Major
About this trial
This is an interventional treatment trial for β-thalassemia Major focused on measuring deferasirox
Eligibility Criteria
Inclusion Criteria:
- Pediatric patients aged from 2 to less than 6 years old.
- Patients with transfusion dependent β-thalassemia major.
- Serum ferritin values ≥ 1000 ng/ml at screening.
- Written informed consent must be obtained from the patient's legal guardian in accordance with local law and regulation prior to any screening procedures.
Exclusion Criteria:
- Non-transfusion-dependent thalassemia.
- Systemic diseases which would prevent study treatment (e.g. uncontrolled hypertension, cardiovascular, renal, hepatic, metabolic, etc.)
- Serum creatinine > age adjusted ULN.
- Significant proteinuria as indicated by a urinary protein/creatinine ratio (UPCR) ≥ 0.5mg/mg in a non-first void urine sample at screening. If UPCR is found to be ≥ 0.5 mg/mg the test can be repeated after 1 month.
- ALT/AST > 2.5xULN and total bilirubin > 1×ULN.
- Left ventricular ejection fraction < 56% by echocardiography.
- Patient has a known history of HIV seropositivity or history of active/treated hepatitis B or C (a test for screening is not required).
- A history of clinically relevant ocular and/or auditory toxicity related to iron chelation therapy
- Any surgical or medical conditions which will significantly alter the absorption, distribution, metabolism or excretion of the drug(e.g. ulcerative disease, uncontrolled nausea, vomiting, malabsorption syndrome, obstruction, or stomach and/or small bowel resection).
- Other conditions which investigator deems potential harm to patients if participate the study.
Sites / Locations
Arms of the Study
Arm 1
Experimental
Deferasirox
The study will provide PK, safety, tolerability and efficacy data collected during 48 weeks of treatment with deferasirox in Chinese pediatric patients with transfusion dependent -thalassemia major, aged 2 to <6 years at enrollment. The target patient pool consists of 20 patients with evidence of iron overload measured by serum ferritin level at the start of study. Patients will start their deferasirox treatment with a dose of 20 mg/kg/day. Serum ferritin will be monitored every month and the dose of deferasirox will be adjusted if necessary every 3 months based on the trends in serum ferritin. Other possible dose adjustments will be based on the patient's safety assessments.