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Venetoclax-Dexamethasone in Relapsed and/or Refractory t(11;14) Amyloidosis

Registration Number
NCT05451771
Lead Sponsor
Rajshekhar Chakraborty, MD
Brief Summary

The purpose of this study is assess safety, safest dose, and effectiveness of venetoclax in combination with dexamethasone in participants with t(11;14) positive relapsed (comes back) or refractory (did not get better) light chain amyloidosis.

Detailed Description

This study is a phase 1/2 study of venetoclax-dexamethasone combination therapy in relapsed/refractory t(11;14) systemic immunoglobulin light chain amyloidosis (AL) amyloidosis. The phase 1 is a dose escalation designed to determine the maximum tolerated dose (MTD) and the recommended phase 2 dose (RP2D) of venetoclax in combination with low-dose weekly dexamethasone. There will be four candidate-dosing cohorts of venetoclax with or without dexamethasone in the Phase I dose-escalation. Dose escalation will be guided by the Bayesian optimal interval (BOIN) design with accelerated titration up to a total sample size of 15 participants.

The phase 2 portion is a randomized open-label study comparing the MTD or RP2D of venetoclax in combination with dexamethasone versus investigator's choice (daratumumab, pomalidomide, bendamustine, or ixazomib (with or without dexamethasone).

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
53
Inclusion Criteria
  • Age ≥ 18 years at time of signing Informed Consent Form
  • Ability to comply with the study protocol, in the investigator's judgment
  • Confirmed diagnosis of systemic AL amyloidosis by mass spectrometry or immunohistochemistry (IHC) on a tissue biopsy
  • Has received ≥1 prior lines of therapy, including an anti-cluster of differentiation 38 (CD 38) monoclonal antibody
  • Participants with a history of autologous hematopoietic cell transplantation must have recovered from any transplant-related toxicities
  • Presence of t(11;14) on FISH at any time since diagnosis (Eligibility must confirmed by FISH testing at Columbia University Irving Medical Center (CUIMC)
  • Eastern Cooperative Oncology Group (ECOG) performance status of 0, 1, or 2
Exclusion Criteria
  • Known hypersensitivity to any of the study drugs
  • History of other malignancy that could affect compliance with the protocol or interpretation of results (Patients with a history of curatively treated basal or squamous cell carcinoma of the skin, in situ carcinoma of the cervix, breast cancer, or Hodgkin's Lymphoma are generally eligible. Patients with a malignancy that has been treated, but not with curative intent, will be excluded, unless the malignancy has been in remission without treatment for ≥ 2 years prior to enrollment.)
  • Evidence of other clinically significant uncontrolled condition(s) including, but not limited to, uncontrolled systemic infection (viral, bacterial, or fungal)
  • Patients on renal replacement therapy
  • Known GI disease or GI procedure that could interfere with oral absorption (including difficulty swallowing)
  • New York Heart Association (NYHA) Class III or IV heart failure
  • Mayo stage three-B (IIIB) with N-terminal pro-hormone B-type natriuretic peptide (NT-Pro BNP) > 8500 pg/mL
  • Prior exposure to anti-apoptotic protein B-cell lymphoma 2 (BCL-2) inhibitors
  • Patients with human immunodeficiency virus (HIV) who are not on highly active antiretroviral therapy (HAART) or those with active hepatitis A, B, or C infection
  • Patients meeting criteria for symptomatic multiple myeloma by one of the following:(a) Lytic lesions on imaging (b) Plasmacytoma, (c) Hypercalcemia without any alternate etiology, or (c) Bone marrow plasma cell infiltrate of greater than 60%

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Phase 1: Venetoclax 400mgVenetoclax Oral Tablet, 400 mgCohort 2: Venetoclax 400 mg tablet, once daily for up to 2 cycles after achieving best response, for a maximum of 6 cycles (1 cycle = 28 days)
Phase 1: Venetoclax 400mg + Dexamethasone 10 mgVenetoclax Oral Tablet, 400 mgCohort 3: Venetoclax 400 mg tablet, once daily and Dexamethasone 10 mg tablet once weekly, for up to 2 cycles after achieving best response, for a maximum of 6 cycles (1 cycle = 28 days)
Phase 1: Venetoclax 400mg + Dexamethasone 20 mgVenetoclax Oral Tablet, 400 mgCohort 4: Venetoclax 400 mg tablet, once daily and Dexamethasone 20 mg tablet once weekly, for up to 2 cycles after achieving best response, for a maximum of 6 cycles (1 cycle = 28 days)
Phase 1: Venetoclax 200 mgVenetoclax Oral Tablet, 200 mgCohort 1: Venetoclax 200 mg tablet, once daily for up to 2 cycles after achieving best response, for a maximum of 6 cycles (1 cycle = 28 days)
Phase 1: Venetoclax 200 mgFISH assayCohort 1: Venetoclax 200 mg tablet, once daily for up to 2 cycles after achieving best response, for a maximum of 6 cycles (1 cycle = 28 days)
Phase 1: Venetoclax 400mgFISH assayCohort 2: Venetoclax 400 mg tablet, once daily for up to 2 cycles after achieving best response, for a maximum of 6 cycles (1 cycle = 28 days)
Phase 1: Venetoclax 400mg + Dexamethasone 10 mgFISH assayCohort 3: Venetoclax 400 mg tablet, once daily and Dexamethasone 10 mg tablet once weekly, for up to 2 cycles after achieving best response, for a maximum of 6 cycles (1 cycle = 28 days)
Phase 1: Venetoclax 400mg + Dexamethasone 10 mgDexamethasone Oral, 10 mgCohort 3: Venetoclax 400 mg tablet, once daily and Dexamethasone 10 mg tablet once weekly, for up to 2 cycles after achieving best response, for a maximum of 6 cycles (1 cycle = 28 days)
Phase 1: Venetoclax 400mg + Dexamethasone 20 mgFISH assayCohort 4: Venetoclax 400 mg tablet, once daily and Dexamethasone 20 mg tablet once weekly, for up to 2 cycles after achieving best response, for a maximum of 6 cycles (1 cycle = 28 days)
Phase 1: Venetoclax 400mg + Dexamethasone 20 mgDexamethasone Oral, 20 mgCohort 4: Venetoclax 400 mg tablet, once daily and Dexamethasone 20 mg tablet once weekly, for up to 2 cycles after achieving best response, for a maximum of 6 cycles (1 cycle = 28 days)
Phase 2: Venetoclax MTD with DexamethasoneVenetoclax MTD with DexamethasoneVenetoclax MTD (200 mg or 400 mg) with Dexamethasone (10 mg or 20 mg) as determined by the phase I results
Phase 2: Control Arm (Investigator's Choice)Dexamethasone Oral, 20 mgParticipants will receive one of the following as determined by the investigator: Daratumumab, Pomalidomide, Bendamustine, or Ixazomib (+/- dexamethasone)
Phase 2: Control Arm (Investigator's Choice)Daratumumab InjectionParticipants will receive one of the following as determined by the investigator: Daratumumab, Pomalidomide, Bendamustine, or Ixazomib (+/- dexamethasone)
Phase 2: Control Arm (Investigator's Choice)PomalidomideParticipants will receive one of the following as determined by the investigator: Daratumumab, Pomalidomide, Bendamustine, or Ixazomib (+/- dexamethasone)
Phase 2: Control Arm (Investigator's Choice)BendamustineParticipants will receive one of the following as determined by the investigator: Daratumumab, Pomalidomide, Bendamustine, or Ixazomib (+/- dexamethasone)
Phase 2: Control Arm (Investigator's Choice)IxazomibParticipants will receive one of the following as determined by the investigator: Daratumumab, Pomalidomide, Bendamustine, or Ixazomib (+/- dexamethasone)
Primary Outcome Measures
NameTimeMethod
Hematologic ≥ Very Good Partial Response (VGPR) Rate (Phase 2)Up to 6 cycles (approximately 6 months)

Hematologic ≥VGPR rate defined as proportion of participants achieving VGPR, low serum differential free light chain concentration (dFLC) partial response (PR), or a complete (CR).

VGPR is defined as the difference between involved and uninvolved free light chain (FLC) \[dFLC\] \< 40 mg/L. Low dFLC PR is defined as achieving a dFLC\<10 mg/L, low dFLC PR will be considered as a deep hematologic response and included in the ≥VGPR category). CR is defined as negative serum and urine immunofixation electrophoresis along with a serum free light chain ratio that lies within the normal range or skewed towards the non-amyloid forming light chain, as per institutional laboratory values

Number of Participants with Dose Limiting Toxicities (DLT) (Phase 1)Up to 6 cycles (approximately 6 months)

The number of participants with dose limiting toxicities for each treatment dose will be used to determine the MTD. Dose limiting toxicity defined as grade 4 neutropenia lasting more than 5 days, any grade febrile neutropenia, grade 4 thrombocytopenia, grade 3 thrombocytopenia with bleeding, other therapy related non-hematologic toxicity of grade 2 or higher that requires discontinuation of therapy, clinical tumor lysis syndrome (TLS), laboratory TLS if the metabolic abnormalities are considered clinically significant by the investigator. All other grade 3 or higher adverse events (AEs) will be considered as DLTs with a few exceptions.

Secondary Outcome Measures
NameTimeMethod
Overall Hematologic Response Rate (HRR) (Phase 2)Up to 1 year

HRR defined as proportion of patients achieving partial response (PR) or better

Duration of Hematologic Response (DOHR) (Phase 2)Up to 1 year

DOHR defined as time from the date of first documentation of hematologic response to the date of first documented hematologic disease progression

Overall Organ Response Rate (ORR) (Phase 2)Up to 1 year

ORR defined as proportion of evaluable participants achieving organ response in each involved organ

Progression Free Survival (PFS) (Phase 2)Up to 1 year

PFS defined as time from the date of enrollment to hematologic progression or death, whichever is earlier

Time to hematologic ≥VGPR (Phase 2)Up to 1 year

Time to hematologic ≥VGPR defined as time from the first dose of study treatment to achievement of deep hematologic response (VGPR, low dFLC PR, or a CR)

Time to next treatment (TTNT) (Phase 2)Up to 1 year

TTNT defined as time from the date of enrollment to initiation of a subsequent line of therapy

Major Organ Deterioration-Progression Free Survival (MOD-PFS) (Phase 2)Up to 1 year

MOD-PFS defined as time from the date of enrollment to one of the following events (whichever occurs first): death, clinical manifestation of cardiac/renal failure, or development of hematologic progression of disease

Patient-reported outcomes (PROs) (Phase 2)Start of each cycle (Day 1 of each 28 day cycle) and Follow-up (every 8 weeks for up to 1 year)

PROs defined as longitudinal score of "Physical Functioning" domain of Patient-Reported Outcomes Measurement Information System (PROMIS)-29 Profile v2.0 The Physical Functioning domain contains four items with a 1 (unable to do) to 5 (without any difficulty) numeric rating.

Overall Survival (OS) (Phase 2)Up to 1 year

OS defined as the time from the date of enrollment to death from any cause

Trial Locations

Locations (3)

New York Presbyterian Hospital/Columbia University Irving Medical Center

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New York, New York, United States

Mayo Clinic Rochester

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Rochester, Minnesota, United States

Froedtert Hospital & the Medical College of Wisconsin

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Milwaukee, Wisconsin, United States

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