Bortezomib for Immunoglobulin Light Chain(AL) Amyloidosis

Study Overview
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Brief Summary
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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.

Detailed Description
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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.

Official Title
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Bortezomib With Dexamethasone as the First-line Treatment for Immunoglobulin Light Chain(AL) Amyloidosis

Conditions
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Amyloidosis

Intervention / Treatment
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* Drug: bortezominb and dexamethasone


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Female or male patients aged between 18 to 80 years Renal or other type biopsy was used to diagnose AL amyloidosis Understand and voluntarily sign an informed consent form ECOG score 0-3 points Adequate residual organ function Exclusion Criteria: Receiving any other investigational agent which would be considered as a treatment for the primary neoplasm Grade 3 sensory or grade 1 painful peripheral neuropathy Known hypersensitivity to bortezomib, boron or mannitol Cardiac syncope, uncompensated New York Heart Association (NYHA) Class 3 or 4 congestive heart failure Clinically overt multiple myeloma Pregnant or nursing women.

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| bortezominb and dexamethasone group<br> | Drug: bortezominb and dexamethasone<br>* 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.<br>* Other names: Velcade;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Hematologic response rate | Hematologic responses were evaluated according to the novel criteria of the International Society of Amyloidosis | 3 months | 

Secondary Outcome Measures

| Outcome Measure | 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 | 
| Progression free survival |  | From date of the start of treatment to date of disease progression,up to 3 months |