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AL amyloidosis

AL Amyloidosis is caused by the accumulation of an immunoglobulin light chain protein

Prevelance

30 / 100,000

33,100 - 165,500

US Estimated

51,350 - 256,750

Europe Estimated

Age of Onset

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ICD-10

E85.9

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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5 Facts you should know

FACT

1

In AL amyloidosis, light chains come together to form amyloid deposits, which can cause serious damage to different organs

FACT

2

The kidneys are the most commonly affected organ in AL amyloidosis

FACT

3

Heart complications, which affect more than a third of AL patients, include heart failure and irregular heart beat

FACT

4

The most effective treatment is autologous bone marrow transplants with stem cell rescues

FACT

5

Two thirds of patients with AL amyloidosis are male and less than 5% of patients are under 40 years of age

AL amyloidosis is also know as...

AL amyloidosis

Primary systemic amyloidosis; Amyloidosis AL; Light chain amyloidosis; Systemic AL amyloidsis; Primary AL amyloidosis

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Which organ is most commonly affected by AL amyloidosis?

Common signs & symptoms

At the time this analysis was conducted, there were no common signs or symptoms for this disease.

Current treatments

The resources below provide information about treatment options for this condition. If you have questions about which treatment is right for you, talk to your healthcare professional.

Management Guidelines

Top Clinical Trials

TitleDescriptionPhasesStatusInterventionsMore Information
Propylene Glycol-Free Melphalan Hydrochloride (Evomela) in AL Amyloidosis PatientsThis is a single arm, open label study designed to evaluate the safety and efficacy of propylene glycol-free melphalan hydrochloride in patients with AL amyloidosis. Treatment will be comprised of propylene glycol-free melphalan hydrochloride administered intravenously at a dose of 70-100 mg/m2/day on Days -3 and -2 as conditioning prior to autologous stem cell transplantation.Phase 2RecruitingDrug: Propylene Glycol-Free Melphalan HydrochlorideClick here for more information
Phase 1/2 Study of ZN-d5 for the Treatment of Relapsed or Refractory Light Chain (AL) AmyloidosisThis is a single arm, Open-Label, Phase 1/2 Study of ZN-d5 for the Treatment of Relapsed or Refractory Light Chain (AL) Amyloidosis.Phase 1/2RecruitingDrug: ZN-d5Click here for more information
COVID-19 VAX Booster Dosing in Patients With Hematologic MalignanciesTo determine whether protective antibody levels increase after booster dosing with the Moderna COVID-19 vaccine in patients diagnosed with Hematologic Malignancies who have low antibody levels after a prior first vaccination with any of the SARS-CoV2 vaccines that were authorized for use in the USA.Phase 2RecruitingDrug: A single "booster" dose of the Moderna mRNA COVID-19 vaccineClick here for more information
Daratumumab, Pomalidomide, and Dexamethasone (DPd) in Relapsed/Refractory Light Chain Amyloidosis Patients Previously Exposed to DaratumumabThis study will test the hypothesis that in patients with previous daratumumab exposure, combination therapy of daratumumab, pomalidomide, and dexamethasone (DPd) will yield higher complete remission (CR) rates in relapsed/refractory amyloidosis than historical pomalidomide/dexamethasone treatment.Phase 2RecruitingDrug: Daratumumab|Drug: Pomalidomide|Drug: DexamethasoneClick here for more information
Ixazomib In Combination With Cyclophosphamide And Dexamethasone for Newly Diagnosed AL AmyloidosisThe primary purpose of this study is to determine the safest dose of the medications and how well the combination is, or what is the "maximum tolerated dose" (MTD).Phase 1|Phase 2RecruitingDrug: Ixazomib|Drug: Cyclophosphamide|Drug: DexamethasoneClick here for more information
A Study to Evaluate the Effectiveness and Safety of CAEL-101 in Patients With Mayo Stage IIIa AL AmyloidosisThe primary purpose of this study is to determine if CAEL-101 improves the overall survival in Patients with cardiac AL Amyloidosis.Phase 3RecruitingDrug: CAEL-101|Other: Placebo|Drug: cyclophosphamide, bortezomib, and dexamethasone (CyBorD) regimenClick here for more information
A Study to Evaluate the Effectiveness and Safety of CAEL-101 in Patients With Mayo Stage IIIb AL AmyloidosisThe primary purpose of this study is to determine if CAEL-101 improves the overall survival in Patients with cardiac AL Amyloidosis.Phase 3RecruitingDrug: CAEL-101|Other: Placebo|Drug: cyclophosphamide, bortezomib, and Dexamethasone (CyBorD) regimenClick here for more information
Ixazomib Maintenance Study in Patients With AL AmyloidosisThe purpose of this study is to learn if Ixazomib maintenance treatment (chemotherapy) works to control the disease.Phase 2RecruitingDrug: Ixazomib|Drug: DexamethasoneClick here for more information
APG-2575 in Combination With Novel Therapeutic Regimens in Subjects With Relapsed or Refractory Multiple MyelomaThis is a Phase Ib/II, open-label, multi-center study evaluating the safety, tolerability, efficacy, and PK/ Pharmacodynamics of APG-2575 in combination with Pd/DRd in patients with relapsed/refractory (RR) multiple myeloma (MM)Phase 1/2RecruitingDrug: APG-2575+ Pd|Drug: APG-2575 + DRdClick here for more information
A Study to Evaluate the Efficacy and Safety of Birtamimab in Mayo Stage IV Patients With AL AmyloidosisA Phase 3 study to evaluate the efficacy and safety of birtamimab plus standard of care compared to placebo plus standard of care in patients with AL amyloidosis in Mayo Stage IV.Phase 3RecruitingDrug: Birtamimab|Other: Placebo|Drug: Standard of Care ChemotherapyClick here for more information

Top Treatments in Research

AgentClass/Mechanism of ActionDevelopment StatusCompanyClinical StudiesMore Information
propylene glycol-free melphalan hydrochloridePropylene Glycol-Free melphalan HCL for Injection (PGF-Mel) is a new formulation that incorporates Captisol, a specially modified cyclodextrin, to improve melphalan stability.Phase 2Spectrum PharamceuticalsClick here for more informationClick here for more information
ZN-d5A potent, selective, and orally bioavailable BCL-2 inhibitorPhase 1/2K-Group Alpha, Inc.Click here for more informationClick here for more information
Moderna COVID-19 vaccinemRNA vaccinePhase 2ModernaClick here for more informationClick here for more information
Daratumumab/Pomalidomide/DexamethasoneDaratumumab is a human mab that binds to a unique cd38 epitope on cd38-expressing cells. Pomalidomide is an immunomodulatory and antineoplastic agent.Phase 2Weill Medical College of Cornell UniversityClick here for more informationClick here for more information
Ixazomib/Cyclophosphamide/DexamethasoneIxazomib is a second-generation proteasome inhibitorPhase 1/2Icahn School of Medicine at Mount Sinai
Collaborator:
Millennium Pharmaceuticals, Inc.
Click here for more informationClick here for more information
CAEL-101CAEL-101 is a monoclonal antibody that acts by binding to amyloid light chain fibrils and promoting their clearance from affected tissuesPhase 3Caelum Biosciences, Inc.Click here for more informationClick here for more information
CAEL-101CAEL-101 is a monoclonal antibody that acts by binding to amyloid light chain fibrils and promoting their clearance from affected tissuesPhase 3Caelum Biosciences, Inc.Click here for more informationClick here for more information
IxazomibIxazomib is a second-generation proteasome inhibitorPhase 2Memorial Sloan Kettering Cancer CenterClick here for more informationClick here for more information
APG-2575+ PdA novel, orally administered Bcl-2‒selective inhibitorPhase 1/2Ascentage Pharma GroupClick here for more informationClick here for more information
BirtamimabBirtamimab (NEOD001), is an investigational monoclonal antibody designed to specifically and selectively target and clear the amyloid that accumulates and causes organ dysfunction and failure in patients with AL amyloidosisPhase 3Prothena BiosciencesClick here for more informationClick here for more information