NCT04504825

Brief Summary

AL (or light chain) amyloidosis begins in the bone marrow where abnormal proteins misfold and create free light chains that cannot be broken down. These free light chains bind together to form amyloid fibrils that build up in the extracellular space of organs, affecting the kidneys, heart, liver, spleen, nervous system and digestive tract. The primary purpose of this study is to determine whether CAEL-101, a monoclonal antibody that removes AL amyloid deposits from tissues and organs, improves overall survival, reduces cardiovascular related hospitalizations and it is safe and well tolerated in patients with stage IIIb AL amyloidosis.

Trial Health

87
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
125

participants targeted

Target at P25-P50 for phase_3

Timeline
18mo left

Started Aug 2020

Longer than P75 for phase_3

Geographic Reach
18 countries

87 active sites

Status
active not recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Progress80%
Aug 2020Oct 2027

First Submitted

Initial submission to the registry

July 20, 2020

Completed
18 days until next milestone

First Posted

Study publicly available on registry

August 7, 2020

Completed
18 days until next milestone

Study Start

First participant enrolled

August 25, 2020

Completed
4.7 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

May 10, 2025

Completed
2.5 years until next milestone

Study Completion

Last participant's last visit for all outcomes

October 22, 2027

Expected
Last Updated

March 17, 2026

Status Verified

March 1, 2026

Enrollment Period

4.7 years

First QC Date

July 20, 2020

Last Update Submit

March 16, 2026

Conditions

Keywords

Plasma Cell Dyscrasiacyclophosphamide, bortezomib and dexamethasone (CyBorD)AL AmyloidosisAmyloid, Light chain Amyloidosistreatment-naïveMayo Stage IIIb

Outcome Measures

Primary Outcomes (2)

  • A hierarchical combination of Time to All-cause Mortality and Frequency of Cardiovascular hospitalizations

    From the date of randomization to date of death or Primary Evaluation Treatment Period (PETP) (up to 51 months)

  • Number of Participants with Treatment Emergent Adverse Events (TEAEs)

    From date of randomization to Primary Evaluation Treatment Period (PETP) (up to 56 months)

Secondary Outcomes (7)

  • Time to All-cause Mortality

    From the date of randomization to date of death or Primary Evaluation Treatment Period (PETP) (up to 51 months)

  • Frequency of cardiovascular-related hospitalizations (CVH)

    From the date of randomization to date of death or Primary Evaluation Treatment Period (PETP) (up to 51 months)

  • Change from Baseline to week 50 in the Kansas City Cardiomyopathy Questionnaire-Overall Score (KCCQ-OS)

    Baseline, Week 50

  • Change from Baseline to Week 50 in N - Terminal Pro-B-type Natriuretic Peptide (NT-proBNP) in blood samples

    Baseline, Week 50

  • Change from Baseline to Week 50 in Cardiac Improvement by Global Longitudinal Strain (GLS%)

    Baseline, Week 50

  • +2 more secondary outcomes

Study Arms (2)

CAEL-101 combined with SoC plasma cell dyscrasia

EXPERIMENTAL

CAEL-101 is administered as an intravenous (IV) infusion over approximately 2 hours. It is planned that all patients will continue their double-blind treatment until the last patient is randomized in the study plus 18 months. The study is divided into 2 parts, the Primary Evaluation Treatment period part and the Open-Label Extension period of Study.

Drug: CAEL-101Drug: cyclophosphamide, bortezomib, and Dexamethasone (CyBorD) regimen

Placebo combined with SoC plasma cell dyscrasia

PLACEBO COMPARATOR

Patients randomized to receive placebo will receive 0.9% normal saline in an equivalent volume to a CAEL-101 infusion (approximately 250 cc). It is planned that all patients will continue their double-blind treatment until the last patient is randomized in the study plus 18 months.

Other: PlaceboDrug: cyclophosphamide, bortezomib, and Dexamethasone (CyBorD) regimen

Interventions

The investigational product, CAEL-101, is formulated as a sterile liquid solution of protein plus excipients for dilution in a single-use, stoppered, glass vial. Each 10 mL vial contains 300 mg of CAEL-101 at a concentration of 30 mg/mL. CAEL-101 will be diluted with commercially available 0.9% Normal Saline.

CAEL-101 combined with SoC plasma cell dyscrasia
PlaceboOTHER

Commercially available 0.9% Normal Saline will be used as the placebo.

Placebo combined with SoC plasma cell dyscrasia

According to institutional standard of care.

CAEL-101 combined with SoC plasma cell dyscrasiaPlacebo combined with SoC plasma cell dyscrasia

Eligibility Criteria

Age18 Years+
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)

You may qualify if:

  • AL amyloidosis stage IIIb based on the European Modification of the 2004 Standard Mayo Clinic Staging (NT-proBNP \> 8,500 ng/L) at the time of Screening
  • Measurable hematologic disease at Screening as defined by at least one of the following:
  • Involved/uninvolved free light chain difference (dFLC) \> 4 mg/dL or
  • Involved free light chain (iFLC) \> 4 mg/dL with abnormal Kappa/Lambda ratio or
  • Serum protein electrophoresis (SPEP) m-spike \> 0.5 g/dL
  • Histopathological diagnosis of amyloidosis based on polarizing light microscopy of green bi-refringent material in Congo red stained tissue specimens AND confirmation of AL derived amyloid deposits by at least one of the following:
  • Immunohistochemistry/Immunofluorescence or
  • Mass spectrometry or
  • Characteristic electron microscopy appearance/Immunoelectron microscopy
  • Cardiac involvement as defined by:
  • Documented clinical signs and symptoms supportive of a diagnosis of heart failure in the setting of a confirmed diagnosis of AL amyloidosis in the absence of an alternative explanation for heart failure AND
  • At least one of the following:
  • i. Endomyocardial biopsy demonstrating AL cardiac amyloidosis or ii. Echocardiogram demonstrating a mean left ventricular wall thickness (calculated as \[IVSd+LPWd\]/2) of \> 12 mm at diastole in the absence of other causes (e.g., severe hypertension, aortic stenosis), which would adequately explain the degree of wall thickening or iii. Cardiac magnetic resonance imaging (MRI) with gadolinium contrast agent diagnostic of cardiac amyloidosis
  • Planned first-line treatment for plasma cell dyscrasia is cyclophosphamide-bortezomib-dexamethasone (CyBorD)-based regimen administered as SoC
  • Women of childbearing potential (WOCBP) must have a negative pregnancy test during Screening and must agree to use highly effective contraception from Screening to at least 5 months following the last study drug administration or 12 months following the last dose of her PCD therapy, whichever is longer
  • +1 more criteria

You may not qualify if:

  • Have any other form of amyloidosis other than AL amyloidosis
  • Received prior therapy for AL amyloidosis or multiple myeloma. A maximum exposure of 2 weeks of a CyBorD-based PCD treatment after Screening laboratory samples are obtained and prior to randomization is allowed
  • Has POEMS (plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes) syndrome or multiple myeloma defined as clonal bone marrow plasma cells \> 10% from a bone marrow biopsy (performed ≤ 3 months prior to signing the ICF) or biopsy-proven (performed ≤ 3 months prior to signing the ICF) bony or extramedullary plasmacytoma AND one or more of the following CRAB features:
  • a. Evidence of end organ damage that can be attributed to the underlying plasma cell proliferative disorder (e.g., multiple myeloma and POEMS syndrome), specifically: i. Hypercalcemia: serum calcium \> 0.25 mmol/L (\> 1 mg/dL) higher than the ULN or \> 2.75 mmol/L (\> 11 mg/dL) OR ii. Renal insufficiency: creatinine clearance \< 40 mL per minute or serum creatinine \> 177 mol/L (\> 2 mg/dL) OR iii. Anemia: hemoglobin value of \> 20 g/L below the lowest limit of normal, or a hemoglobin value \< 100 g/L OR iv. Bone lesions: one or more osteolytic lesion on imaging tests (performed ≤ 3 months prior to signing the ICF): skeletal radiography, CT, or PET/CT, or MRI. If bone marrow has \< 10% clonal plasma cells, more than one bone lesion is required to distinguish from solitary plasmacytoma with minimal marrow involvement OR b. Any one of the following biomarkers of malignancy: i. 60% or greater clonal plasma cells on bone marrow examination OR ii. More than one focal lesion on MRI that is at least 5mm or greater in size
  • Have supine systolic blood pressure \< 90 mmHg or symptomatic orthostatic hypotension, defined as a decrease in systolic blood pressure upon standing of \> 30 mmHg despite medical management (e.g., midodrine, fludrocortisones) in the absence of volume depletion

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (90)

Research Site

Duarte, California, 91010, United States

Location

Research Site

Palo Alto, California, 94304, United States

Location

Research Site

San Francisco, California, 94143, United States

Location

Research Site

Weston, Florida, 33331, United States

Location

Research Site

Indianapolis, Indiana, 46202, United States

Location

Research Site

New Orleans, Louisiana, 70112, United States

Location

Research Site

Boston, Massachusetts, 02111, United States

Location

Research Site

Boston, Massachusetts, 02118, United States

Location

Research Site

Boston, Massachusetts, 02215, United States

Location

Research Site

Detroit, Michigan, 48201, United States

Location

Research Site

Rochester, Minnesota, 55905, United States

Location

Research Site

St Louis, Missouri, 63110, United States

Location

Research Site

New York, New York, 10032, United States

Location

Research Site

New York, New York, 10065, United States

Location

Research Site

Rochester, New York, 14642, United States

Location

Research Site

Winston-Salem, North Carolina, 27157, United States

Location

Research Site

Cleveland, Ohio, 44195, United States

Location

Research Site

Columbus, Ohio, 43210, United States

Location

Research Site

Portland, Oregon, 97239, United States

Location

Research Site

Philadelphia, Pennsylvania, 19104, United States

Location

Research Site

Nashville, Tennessee, 37232, United States

Location

Research Site

Dallas, Texas, 75390, United States

Location

Research Site

Houston, Texas, 77030, United States

Location

Research Site

Salt Lake City, Utah, 84112, United States

Location

Research Site

Seattle, Washington, 98109, United States

Location

Research Site

Milwaukee, Wisconsin, 53226, United States

Location

Research Site

Box Hill, 3128, Australia

Location

Research Site

Brisbane, 4102, Australia

Location

Research Site

Westmead, 2145, Australia

Location

Research Site

Linz, 4020, Austria

Location

Research Site

Vienna, 1090, Austria

Location

Research Site

Anderlecht, 1070, Belgium

Location

Research Site

Leuven, 3000, Belgium

Location

Research Site

Porto Alegre, 90110-270, Brazil

Location

Research Site

Ribeirão Preto, 14048-900, Brazil

Location

Research Site

Salvador, 41253-190, Brazil

Location

Research Site

São José do Rio Preto, 15090-000, Brazil

Location

Research Site

Calgary, Alberta, T2N 4N2, Canada

Location

Research Site

Edmonton, Alberta, T6G 1Z2, Canada

Location

Research Site

Toronto, Ontario, M5G 2M9, Canada

Location

Research Site

Beijing, 100730, China

Location

Research Site

Guangzhou, 510180, China

Location

Research Site

Hangzhou, 310009, China

Location

Research Site

Wenzhou, 325000, China

Location

Research Site

Wuhan, 430022, China

Location

Research Site

Ostrava - Poruba, 70852, Czechia

Location

Research Site

Prague, 12808, Czechia

Location

Research Site

Caen, 14033, France

Location

Research Site

Créteil, 94000, France

Location

Research Site

Limoges, 87042, France

Location

Research Site

Marseille, 13009, France

Location

Research Site

Paris, 75010, France

Location

Research Site

Pessac, 33604, France

Location

Research Site

Pierre-Bénite, 69310, France

Location

Research Site

Poitiers, 86021, France

Location

Research Site

Rennes, 35033, France

Location

Research Site

Toulouse, 31100, France

Location

Research Site

Tours, 37044, France

Location

Research Site

Berlin, 13353, Germany

Location

Research Site

Düsseldorf, 40225, Germany

Location

Research Site

Essen, 45147, Germany

Location

Research Site

Hamburg, 22767, Germany

Location

Research Site

Heidelberg, 69120, Germany

Location

Research Site

Würzburg, 97080, Germany

Location

Research Site

Athens, 11528, Greece

Location

Research Site

Rio, 26504, Greece

Location

Research Site

Haifa, 31096, Israel

Location

Research Site

Jerusalem, 91120, Israel

Location

Research Site

Tel Litwinsky, 52621, Israel

Location

Research Site

Naples, 80131, Italy

Location

Research Site

Pavia, 27100, Italy

Location

Research Site

Pisa, 56126, Italy

Location

Research Site

Roma, 00128, Italy

Location

Research Site

Fukushima, 960-1295, Japan

Location

Research Site

Nagoya, 467-8602, Japan

Location

Research Site

Shibuya-ku, 150-8935, Japan

Location

Research Site

Groningen, 9713 GZ, Netherlands

Location

Research Site

Utrecht, 3508 GA, Netherlands

Location

Research Site

Gdansk, 80-214, Poland

Location

Research Site

Poznan, 60-569, Poland

Location

Research Site

Warsaw, 01-748, Poland

Location

Research Site

Seoul, 03080, South Korea

Location

Research Site

Seoul, 06351, South Korea

Location

Research Site

Barcelona, 08036, Spain

Location

Research Site

Barcelona, 8035, Spain

Location

Research Site

Gijón, 33394, Spain

Location

Research Site

Majadahonda, 28222, Spain

Location

Research Site

Pamplona, 31008, Spain

Location

Research Site

Seville, 41013, Spain

Location

Research Site

London, WC1E 6AG, United Kingdom

Location

MeSH Terms

Conditions

Immunoglobulin Light-chain AmyloidosisParaproteinemias

Interventions

CyclophosphamideBortezomibDexamethasoneClinical Protocols

Condition Hierarchy (Ancestors)

Neoplasms, Plasma CellNeoplasms by Histologic TypeNeoplasmsAmyloidosisProteostasis DeficienciesMetabolic DiseasesNutritional and Metabolic DiseasesLymphoproliferative DisordersImmunoproliferative DisordersImmune System DiseasesBlood Protein DisordersHematologic DiseasesHemic and Lymphatic Diseases

Intervention Hierarchy (Ancestors)

Phosphoramide MustardsNitrogen Mustard CompoundsMustard CompoundsHydrocarbons, HalogenatedHydrocarbonsOrganic ChemicalsPhosphoramidesOrganophosphorus CompoundsBoronic AcidsAcids, NoncarboxylicAcidsInorganic ChemicalsBoron CompoundsPyrazinesHeterocyclic Compounds, 1-RingHeterocyclic CompoundsPregnadienetriolsPregnadienesPregnanesSteroidsFused-Ring CompoundsPolycyclic CompoundsSteroids, FluorinatedTherapeuticsEpidemiologic Study CharacteristicsHealth Care Evaluation MechanismsQuality of Health CareHealth Care Quality, Access, and Evaluation

Study Officials

  • Scott Swenson, MD

    Alexion, AstraZeneca Rare Disease

    STUDY DIRECTOR

Study Design

Study Type
interventional
Phase
phase 3
Allocation
RANDOMIZED
Masking
QUADRUPLE
Who Masked
PARTICIPANT, CARE PROVIDER, INVESTIGATOR, OUTCOMES ASSESSOR
Masking Details
This is a double-blind, randomized, multicenter international Phase 3 study.
Purpose
TREATMENT
Intervention Model
PARALLEL
Model Details: This is a double-blind, randomized, multicenter, international Phase 3 study of CAEL-101 combined with SoC PCD treatment versus placebo combined with SoC PCD treatment in Mayo stage IIIb PCD treatment-naïve AL amyloidosis patients. Approximately 124 patients will be enrolled using a 2:1 randomization ratio and stratification will be based on geographic region across investigator sites. The primary evaluation treatment period (PETP) part of the study will stop when the last patient is randomized in the PETP plus 18 months. Patients in both study intervention groups will be followed from randomization until death from any cause, heart transplant, left valve assist device (LVAD) implantation or until the end of study.
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

July 20, 2020

First Posted

August 7, 2020

Study Start

August 25, 2020

Primary Completion

May 10, 2025

Study Completion (Estimated)

October 22, 2027

Last Updated

March 17, 2026

Record last verified: 2026-03

Data Sharing

IPD Sharing
Will share

Each patient will be assigned a unique identifier after signing the Informed Consent Form (ICF). Patient numbers will not be reassigned. Any patient records or datasets transferred to the Sponsor must contain only the unique identifier and must not include patient names or any information which would make the patient identifiable. Patients will be informed that their personal study-related data will be used by the Sponsor in accordance with local data protection laws and that their medical records may be examined by representatives of the Sponsor, Institutional Review Board (IRB)/Independent Ethics Committee (IEC) members and by inspectors from regulatory authorities. Study monitors will inspect all documents and records that are required to be maintained by the Investigator for this study.

Locations