Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
The study start on January 18, 2017. The Severe(FⅧ<1%) and moderate hemophilia A (FⅧ1%~5%)children with high titer inhibitor(historical peak inhibitor titer≥5BU ) combining with poor ITI risk(s) were enrolled. The low-dose ITI was alone or combined with immunosuppression.
Poor risk(s) includes:①peak historical inhibitor titer≥200BU ②inhibitor titer≥10BU before ITI initiation ③peak inhibitor titer during ITI≥200BU ④time to titer decline to<10BU before ITI≥24 months ⑤age≥8 years at start of ITI ⑥ITI initiated ≥5 years after inhibitor diagnosis ⑦interruptions in ITI≥2 weeks in duration. The low-dose ITI strategy consist of FⅧ(25-50IU/kg)alone or combining with immunosuppression: prednisone and Rituximab when the inhibitor titer ≥40BU ml/ml before or during ITI.
Not provided
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| ITI strategy | Experimental | The low-dose ITI was Coagulation Factor VIII (50IU/kg, every other day) alone or combined with prednisone (2mg Kg-1/day, one month, then taper in three months) depending on the tendency of inhibitor, and Rituximab (375mg/square meter every week for 4 weeks) when the inhibitor titer ≥40BU/ml before or during ITI.Inhibitor and hemorrhage should be retested and recorded periodically. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Coagulation Factor VIII | Drug | Domastic plasma derived factor FVIII (with intermidiate vWF) 50IU/kg every other day |
|
| Measure | Description | Time Frame |
|---|---|---|
| Success rate | Success rate | 2 years |
| Measure | Description | Time Frame |
|---|---|---|
| Annualized Bleeding Rate | How many times for all types of bleeding | 2 year |
| Annualized Joint Bleeding Rate | How many times for joint bleeding |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Not provided
Not provided
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Xin Ni, master | Contact | 010-59616643 | nixin@bch.com.cn |
Not provided
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Beijing Children's Hospital | Recruiting | Beijing | Beijing Municipality | 100045 | China |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 40286913 | Derived | Li Z, Tang Y, Chen Z, Liu G, Yao W, Li G, Cheng X, Peng Y, Cai S, Cui C, Ai D, Zhang J, Poon MC, Zhang W, Wu R. Outcomes of immune tolerance induction with rituximab to eradicate high-titer inhibitor of hemophilia A: depicted by exponential decay model and the gene expression profile of different outcomes by RNA-sequencing. J Thromb Haemost. 2025 Aug;23(8):2436-2448. doi: 10.1016/j.jtha.2025.04.015. Epub 2025 Apr 24. |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| ID | Term |
|---|---|
| D005169 | Factor VIII |
| D011241 | Prednisone |
| D000069283 | Rituximab |
| ID | Term |
|---|---|
| D001779 | Blood Coagulation Factors |
| D001798 | Blood Proteins |
| D011506 | Proteins |
| D000602 | Amino Acids, Peptides, and Proteins |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Prednisone | Drug | 2mg/kg every day for 4 weeks then typering in 3 months |
|
| Rituximab | Drug | 375mg/Square meter for consecutive 4 months |
|
| 2 year |
| Success time | How long to success | 2 years |
| D011498 |
| Protein Precursors |
| D001685 | Biological Factors |
| D011244 | Pregnadienediols |
| D011245 | Pregnadienes |
| D011278 | Pregnanes |
| D013256 | Steroids |
| D000072473 | Fused-Ring Compounds |
| D011083 | Polycyclic Compounds |
| D058846 | Antibodies, Monoclonal, Murine-Derived |
| D000911 | Antibodies, Monoclonal |
| D000906 | Antibodies |
| D007136 | Immunoglobulins |
| D007162 | Immunoproteins |
| D012712 | Serum Globulins |
| D005916 | Globulins |