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Although the use of bortezomib has reported efficacy in amyloid light chain (AL) amyloidosis, the role of bortezomib with dexamethasone (BD) in the first-line treatment of patients with AL amyloidosis should be determined. In this study, the investigators evaluated the efficacy and safety of BD as the first-line treatment of patients with AL amyloidosis.
This study included patients with newly diagnosed AL amyloidosis who were treated with a BD regimen in Jinling hospital. The AL amyloidosis diagnosis was confirmed by renal biopsy, and the assessment of organ involvement were based on consensus criteria. The clinical and laboratory data were collected at the beginning of each cycle and every 3 months thereafter. The initial BD regimen included bortezomib (1.3 mg/m2 i.v.) and dexamethasone (40 mg i.v.) twice weekly for 2 weeks (days 1, 4, 8, and 11), which was followed by a 10-day rest period (days 12-21). The investigators evaluated the efficacy and safety of BD as the first-line treatment of patients with AL amyloidosis.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| bortezominb and dexamethasone group |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| bortezominb and dexamethasone | Drug | The initial BD regimen included bortezomib (1.3 mg/m2 i.v.) and dexamethasone (40 mg i.v.) twice weekly for 2 weeks (days 1, 4, 8, and 11), which was followed by a 10-day rest period (days 12-21). The dose of bortezomib and dexamethasone can be adjusted according to the patients' condition. |
| Measure | Description | Time Frame |
|---|---|---|
| Hematologic response rate | Hematologic responses were evaluated according to the novel criteria of the International Society of Amyloidosis | 3 months |
| Measure | Description | Time Frame |
|---|---|---|
| organ response rate | organ responses were evaluated according to the novel criteria of the International Society of Amyloidosis | 12 months |
| Overall survival | From date of the start of treatment to date of death,up to 3 months |
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Inclusion Criteria:
Exclusion Criteria:
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Patients from hospital,who were diagnosed AL amyloidosis by renal or other type biopsy.
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| ID | Term |
|---|---|
| D000686 | Amyloidosis |
| ID | Term |
|---|---|
| D057165 | Proteostasis Deficiencies |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
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| ID | Term |
|---|---|
| D003907 | Dexamethasone |
| D000069286 | Bortezomib |
| ID | Term |
|---|---|
| D011246 | Pregnadienetriols |
| D011245 | Pregnadienes |
| D011278 | Pregnanes |
| D013256 | Steroids |
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|
|
| Progression free survival | From date of the start of treatment to date of disease progression,up to 3 months |
| D000072473 |
| Fused-Ring Compounds |
| D011083 | Polycyclic Compounds |
| D013259 | Steroids, Fluorinated |
| D001897 | Boronic Acids |
| D000148 | Acids, Noncarboxylic |
| D000143 | Acids |
| D007287 | Inorganic Chemicals |
| D001896 | Boron Compounds |
| D009930 | Organic Chemicals |
| D011719 | Pyrazines |
| D006573 | Heterocyclic Compounds, 1-Ring |
| D006571 | Heterocyclic Compounds |