MCID: IMM246
MIFTS: 57

Immunoglobulin Light Chain Amyloidosis

Categories: Blood diseases, Bone diseases, Cardiovascular diseases, Endocrine diseases, Gastrointestinal diseases, Genetic diseases, Metabolic diseases, Nephrological diseases, Neuronal diseases, Rare diseases
Data Licensing
For inquiries, contact:

Aliases & Classifications for Immunoglobulin Light Chain Amyloidosis

MalaCards integrated aliases for Immunoglobulin Light Chain Amyloidosis:

Name: Immunoglobulin Light Chain Amyloidosis 11 14
Primary Amyloidosis 19 58 71 33
Al Amyloidosis 11 19 58 33
Primary Systemic Amyloidosis 19 58 71
Light Chain Amyloidosis 11 19
Hereditary Amyloid Nephropathy Due to Lysozyme Variant 58
Familial Amyloid Nephropathy Due to Lysozyme Variant 58
Hereditary Renal Amyloidosis Due to Lysozyme Variant 58
Familial Renal Amyloidosis Due to Lysozyme Variant 58
Immunoglobulin Light-Chain Amyloidosis 43
Immunoglobulin Deposition Disease 71
Primary Systemic Al Amyloidosis 19
Amyloidosis Primary Systemic 19
Immunoglobulinic Amyloidosis 33
Systemic Al Amyloidosis 58
Light-Chain Amyloidosis 58
Primary Al Amyloidosis 19
Systemic Al Amyloidsis 19
Lysozyme Amyloidosis 58
Amyloidosis Primary 53
Alys Amyloidosis 58
Amyloidosis Al 19
Amyloid Al 33

Characteristics:


Inheritance:

Alys Amyloidosis: Autosomal dominant 58

Prevelance:

Primary Systemic Amyloidosis: 1-5/10000 (Europe) 58
Al Amyloidosis: 1-9/100000 (Germany, Worldwide, Worldwide, Europe, Europe, Austria, Austria, Belgium, Bulgaria, Bulgaria, Croatia, Croatia, Cyprus, Czech Republic, Czech Republic, Denmark, Denmark, Estonia, Estonia, Finland, Finland, France, France, Germany, Greece, Greece, Hungary, Hungary, Iceland, Ireland, Italy, Italy, Latvia, Latvia, Liechtenstein, Liechtenstein, Lithuania, Lithuania, Luxembourg, Malta, Malta, Netherlands, Netherlands, Norway, Norway, Poland, Poland, Portugal, Portugal, Romania, Romania, Slovakia, Slovenia, Slovenia, Spain, Spain, Sweden, Sweden, United Kingdom, United Kingdom, Brazil, Canada, Canada, Japan, Japan, Russian Federation, Korea, Republic of, Taiwan, Province of China, Taiwan, Province of China, United States) 1-9/1000000 (Belgium, Cyprus, Iceland, Ireland, Luxembourg, Slovakia, Brazil, Russian Federation, Korea, Republic of, United States) 58
Alys Amyloidosis: <1/1000000 (Worldwide) 58

Age Of Onset:

Al Amyloidosis: Adult 58

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare renal diseases
Rare systemic and rhumatological diseases
Rare haematological diseases


Summaries for Immunoglobulin Light Chain Amyloidosis

GARD: 19 AL amyloidosisis the most common form of amyloidosis, a group of disorders in which an abnormal protein called amyloid builds up in tissues and organs. The signs and symptoms of AL amyloidosis vary among patients because the build up may occur in the tongue, intestines, muscles, joints, nerves, skin, ligaments, heart, liver, spleen, or kidneys. To diagnose AL amyloidosis, healthcare professionals use blood or urine tests to identify signs of amyloid protein and a biopsy to confirm the diagnosis.

MalaCards based summary: Immunoglobulin Light Chain Amyloidosis, also known as primary amyloidosis, is related to amyloid tumor and nephrotic syndrome. An important gene associated with Immunoglobulin Light Chain Amyloidosis is LYZ (Lysozyme), and among its related pathways/superpathways are Embryonic and Induced Pluripotent Stem Cells and Lineage-specific Markers and Mesenchymal Stem Cells and Lineage-specific Markers. The drugs Thalidomide and Angiogenesis Inhibitors have been mentioned in the context of this disorder. Affiliated tissues include liver, spleen and kidney, and related phenotypes are fatigue and proteinuria

Orphanet 58 Al amyloidosis: A clonal B-cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains. It usually presents as systemic AL amyloidosis with involvement of one or more parenchymal organ(s) and, less frequently, as localized amyloidosis with usually nodular deposits restricted to a single organ and/or system.

Alys amyloidosis: A rare, hereditary amyloidosis with primary renal involvement characterized by amyloid deposition in the kidney glomeruli and medulla, gastrointestinal tract, liver, spleen and slow disease progression. Symptoms and signs include nausea, vomiting, dyspepsia, gastritis, gastrointestinal hemorrhage, abdominal pain, hepatic rupture, sicca syndrome, purpura and petechiae, lymphadenopathy and renal dysfunction.

Disease Ontology: 11 An amyloidosis that is characterized by misfolded and aggregated amyloidogenic immunoglobulin light chains produced by marrow clonal plasma cells.

Related Diseases for Immunoglobulin Light Chain Amyloidosis

Diseases related to Immunoglobulin Light Chain Amyloidosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 623)
# Related Disease Score Top Affiliating Genes
1 amyloid tumor 30.5 TTR B2M
2 nephrotic syndrome 30.4 TTR GSN F9 B2M APOA1 ALB
3 amyloidosis 30.4 TTR SAA1 NPPB MYOM2 LYZ LECT2
4 plasma cell neoplasm 30.4 SDC1 MYOM2 IGHV4-38-2 CD38 B2M
5 monoclonal gammopathy of uncertain significance 30.3 SDC1 MYOM2 IGHV4-38-2 CD38 B2M ALB
6 marginal zone b-cell lymphoma 30.3 SDC1 IGHV4-38-2 CD38
7 waldenstroem's macroglobulinemia 30.3 SDC1 MYOM2 IGHV4-38-2 CD38 B2M
8 carotenemia 30.2 TTR APCS
9 serum amyloid a amyloidosis 30.2 TTR SAA1 B2M APOA1 ALB
10 chromosome 13q14 deletion syndrome 30.2 IGHV4-38-2 CD38
11 monoclonal paraproteinemia 30.2 SDC1 IGHV4-38-2 B2M
12 lymphoplasmacytic lymphoma 30.1 SDC1 MYOM2 IGHV4-38-2 CD38 B2M
13 dialysis-related amyloidosis 30.1 TTR B2M APCS ALB
14 polyneuropathy 30.1 TTR MYOM2 GSN APOA1 ALB
15 smoldering myeloma 30.1 SDC1 MYOM2 IGHV4-38-2 CD38 B2M
16 immunoglobulin heavy chain amyloidosis 30.1 TTR LECT2 GSN APCS
17 demyelinating polyneuropathy 30.0 TTR MYOM2 ALB
18 intermittent claudication 30.0 TNNT2 B2M ALB
19 primary cutaneous amyloidosis 30.0 TTR GSN APCS
20 gamma heavy chain disease 30.0 SDC1 IGHV4-38-2 CD38
21 cardiac arrest 30.0 TNNT2 TNNI3 NPPB
22 restrictive cardiomyopathy 29.9 TTR TNNT2 TNNT1 TNNI3 NPPB
23 hypothyroidism 29.9 TTR NPPB APOA1 ALB
24 pericarditis 29.9 TNNT2 TNNI3 NPPB
25 amyloid neuropathy 29.9 TTR GSN APOA1
26 deficiency anemia 29.9 TTR F9 CD38 B2M ALB
27 congestive heart failure 29.9 TNNT2 TNNI3 NPPB ALB
28 hereditary amyloidosis 29.8 TTR LYZ GSN B2M APOA1
29 diastolic heart failure 29.8 TTR TNNT2 TNNI3 NPPB
30 angina pectoris 29.8 TNNI3 NPPB APOA1
31 amyloidosis, finnish type 29.7 TTR LECT2 GSN APOA1 APCS
32 aortic valve disease 2 29.7 TTR TNNT2 TNNI3 NPPB
33 wild-type amyloidosis 29.7 TTR NPPB GSN B2M APOA1 APCS
34 respiratory failure 29.7 TTR TNNI3 NPPB ALB
35 lymphoma, mucosa-associated lymphoid type 29.6 SDC1 IGLL5 IGHV4-38-2 B2M
36 amyloidosis, hereditary, transthyretin-related 29.6 TTR TNNT2 NPPB GSN B2M APOA1
37 right bundle branch block 29.6 TNNT2 TNNI3 NPPB
38 cardiac tamponade 29.6 TNNI3 NPPB F9 ALB
39 endocarditis 29.5 TTR TNNT2 TNNI3 ALB
40 constrictive pericarditis 29.5 TTR TNNT2 TNNI3 NPPB ALB
41 atrioventricular block 29.5 TNNT2 TNNI3 NPPB
42 extramedullary plasmacytoma 29.5 SDC1 MYOM2 IGHV4-38-2 CD38 B2M ALB
43 type 1 diabetes mellitus 29.5 NPPB B2M APOA1 ALB
44 pulmonary edema 29.5 TNNT2 TNNI3 NPPB
45 leukemia, chronic lymphocytic 29.4 SDC1 IGLL5 IGHV4-38-2 CD38 B2M
46 systolic heart failure 29.3 TNNT2 TNNI3 NPPB ALB
47 mantle cell lymphoma 29.3 SDC1 IGLL5 IGHV4-38-2 CD38 B2M
48 amyloidosis, familial visceral 29.1 TXNRD3 TTR SAA1 LYZ LECT2 GSN
49 pulmonary embolism 28.9 TNNT2 TNNT1 TNNI3 NPPB F9 ALB
50 heart valve disease 28.9 TTR TNNT2 TNNI3 NPPB MYOM2 ALB

Graphical network of the top 20 diseases related to Immunoglobulin Light Chain Amyloidosis:



Diseases related to Immunoglobulin Light Chain Amyloidosis

Symptoms & Phenotypes for Immunoglobulin Light Chain Amyloidosis

Human phenotypes related to Immunoglobulin Light Chain Amyloidosis:

58 30 (show top 50) (show all 55)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fatigue 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0012378
2 proteinuria 58 30 Frequent (33%) Frequent (79-30%)
HP:0000093
3 hypertrophic cardiomyopathy 58 30 Frequent (33%) Frequent (79-30%)
HP:0001639
4 nephrotic syndrome 58 30 Frequent (33%) Frequent (79-30%)
HP:0000100
5 weight loss 58 30 Frequent (33%) Frequent (79-30%)
HP:0001824
6 renal interstitial amyloid deposits 58 30 Frequent (33%) Frequent (79-30%)
HP:0032613
7 albuminuria 58 30 Frequent (33%) Frequent (79-30%)
HP:0012592
8 monoclonal light chain cardiac amyloidosis 58 30 Frequent (33%) Frequent (79-30%)
HP:0031326
9 increased circulating antibody level 30 Frequent (33%) HP:0010702
10 increased circulating nt-probnp concentration 30 Frequent (33%) HP:0031185
11 increased circulating troponin i concentration 30 Frequent (33%) HP:0410173
12 increased circulating troponin t concentration 30 Frequent (33%) HP:0410174
13 hepatomegaly 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002240
14 renal insufficiency 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000083
15 anemia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001903
16 dyspnea 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002094
17 arrhythmia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0011675
18 bruising susceptibility 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000978
19 hypoalbuminemia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003073
20 constrictive median neuropathy 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0012185
21 obstructive sleep apnea 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002870
22 abdominal distention 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003270
23 xerostomia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000217
24 abnormal salivary gland morphology 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0010286
25 peripheral edema 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0012398
26 nonproductive cough 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0031246
27 abnormal cardiac ventricle morphology 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001713
28 abnormal cardiac atrium morphology 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0005120
29 postural hypotension with compensatory tachycardia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0005307
30 reduced factor x activity 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0008321
31 autonomic erectile dysfunction 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0008652
32 pulmonary interstitial high-resolution computed tomography abnormality 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0025389
33 jaw claudication 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0030164
34 abnormal p wave 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0031595
35 howell-jolly bodies 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0032550
36 elevated circulating alkaline phosphatase concentration 30 Occasional (7.5%) HP:0003155
37 dysphagia 58 30 Very rare (1%) Very rare (<4-1%)
HP:0002015
38 macroglossia 58 30 Very rare (1%) Very rare (<4-1%)
HP:0000158
39 gastrointestinal hemorrhage 58 30 Very rare (1%) Very rare (<4-1%)
HP:0002239
40 hoarse voice 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001609
41 gastroparesis 58 30 Very rare (1%) Very rare (<4-1%)
HP:0002578
42 abnormality of the liver 58 Occasional (29-5%)
43 abnormality of the kidney 58 Frequent (79-30%)
44 peripheral neuropathy 58 Occasional (29-5%)
45 abnormality of the gastrointestinal tract 58 Very rare (<4-1%)
46 increased antibody level in blood 58 Frequent (79-30%)
47 abnormal heart morphology 58 Frequent (79-30%)
48 reduced ejection fraction 58 Excluded (0%)
49 interstitial pulmonary abnormality 58 Frequent (79-30%)
50 elevated alkaline phosphatase 58 Occasional (29-5%)

Drugs & Therapeutics for Immunoglobulin Light Chain Amyloidosis

Drugs for Immunoglobulin Light Chain Amyloidosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 126)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Thalidomide Approved, Investigational, Withdrawn Phase 4 50-35-1 5426
2 Angiogenesis Inhibitors Phase 4
3 Anti-Bacterial Agents Phase 4
4 Anti-Infective Agents Phase 4
5
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
6
Daratumumab Approved Phase 3 945721-28-8
7
Doxycycline Approved, Investigational, Vet_approved Phase 2, Phase 3 564-25-0 54671203
8
Melphalan Approved Phase 3 148-82-3 4053 460612
9
Lenograstim Approved, Investigational Phase 3 135968-09-1
10
D-Phenylalanine Approved, Experimental, Investigational, Nutraceutical Phase 3 63-91-2, 673-06-3 6140 71567
11 Pharmaceutical Solutions Phase 3
12 Antiprotozoal Agents Phase 2, Phase 3
13 Antiparasitic Agents Phase 2, Phase 3
14 Antimalarials Phase 2, Phase 3
15 Adjuvants, Immunologic Phase 3
16 Immunoglobulin G Phase 3
17
Iodine Approved, Investigational Phase 2 7553-56-2 807
18
Cianidanol Approved, Withdrawn Phase 2 154-23-4 9064
19
Silicon Approved, Investigational Phase 2 7440-21-3 4082203
20
Sorbitol Approved, Investigational Phase 2 69-65-8, 50-70-4 453 6251 5780
21
Coenzyme M Approved, Investigational Phase 2 3375-50-6 598 23662354
22
Busulfan Approved, Investigational Phase 2 55-98-1 2478
23
Etoposide Approved Phase 2 33419-42-0 36462
24
Cisplatin Approved Phase 2 15663-27-1 2767 5702198 441203
25
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
26
Fludarabine Approved Phase 1, Phase 2 75607-67-9, 21679-14-1 30751 657237
27
Mycophenolic acid Approved, Investigational Phase 1, Phase 2 24280-93-1 446541
28
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
29
Clotrimazole Approved, Vet_approved Phase 1, Phase 2 23593-75-1 2812
30
Sargramostim Approved, Investigational Phase 2 123774-72-1
31
Lenalidomide Approved Phase 1, Phase 2 191732-72-6 216326
32
Pomalidomide Approved Phase 1, Phase 2 19171-19-8 134780
33
Venetoclax Approved, Investigational Phase 1, Phase 2 1257044-40-8 49846579
34
Edetate calcium disodium anhydrous Approved, Vet_approved Phase 1, Phase 2 62-33-9, 60-00-4 6049
35
Pentetic acid Approved Phase 1, Phase 2 67-43-6
36
Heparin, bovine Approved, Investigational, Withdrawn Phase 1, Phase 2 9005-49-6 22833565 9812414 772
37
Ixazomib Approved, Investigational Phase 1, Phase 2 1072833-77-2 25183872
38
Gliclazide Approved Phase 2 21187-98-4 3475
39
Cyclophosphamide Approved, Investigational Phase 1, Phase 2 50-18-0, 6055-19-2 2907
40
Elotuzumab Approved Phase 2 915296-00-3
41
Sodium citrate Approved, Investigational Phase 1, Phase 2 68-04-2 23431961
42
Siltuximab Approved, Investigational Phase 2 541502-14-1
43
Etanercept Approved, Investigational Phase 2 185243-69-0
44
Amiodarone Approved, Investigational Phase 2 1951-25-3 2157
45
Glycine Approved, Nutraceutical, Vet_approved Phase 1, Phase 2 56-40-6 750
46
Citric acid Approved, Nutraceutical, Vet_approved Phase 1, Phase 2 77-92-9 311
47
Cadexomer iodine Experimental Phase 2 94820-09-4
48
Epicatechin Investigational Phase 2 490-46-0 58571364 72276
49
Molgramostim Investigational Phase 2 99283-10-0
50
Epigallocatechin gallate Investigational Phase 2 989-51-5 65064

Interventional clinical trials:

(show top 50) (show all 140)
# Name Status NCT ID Phase Drugs
1 A Comparative Study of Bortezomib-Thalidomide-Dexamethason and Bortezomib-Cyclophosphamide-Dexamethason in the Treatment of Monoclonal Immunoglobulin Light Chain Amyloidosis: A Prospective Randomized Controlled Trial(BTD-CHINA-TRIAL) Recruiting NCT04612582 Phase 4 Thalidomide;Cyclophosphamide
2 Frontline Lenalidomide for AL Amyloidosis Involving Myocardium: Investigation of Organ Reversing Capacity of Lenalidomide Unknown status NCT04298372 Phase 3 Lenalidomide 25mg
3 Autologous Stem Cell Transplantation (ASCT) Versus Oral Melphalan and High-Dose Dexamethasone in Patients With AL (Primary)Amyloidosis. A Prospective Randomized Trial . Completed NCT00344526 Phase 3 Melphalan;Dexamethasone
4 A Randomized Open-label Multicenter Phase III Trial of Melphalan and Dexamethasone (MDex) Versus Bortezomib, Melphalan and Dexamethasone (BMDex) for Untreated Patients With Systemic Light-chain (AL) Amyloidosis Completed NCT01277016 Phase 3 BMDex
5 Induction Therapy With Bortezomib and Dexamethasone Followed by Autologous Stem Cell Transplantation Versus Autologous Stem Cell Transplantation Alone in the Treatment of AL Amyloidosis Completed NCT01998503 Phase 3 Bortezomib;dexamethasone;Melphalan
6 A Phase 3, Randomized, Controlled, Open-label, Multicenter, Safety and Efficacy Study of Dexamethasone Plus MLN9708 or Physicians Choice of Treatment Administered to Patients With Relapsed or Refractory Systemic Light Chain (AL) Amyloidosis Completed NCT01659658 Phase 3 IXAZOMIB;Dexamethasone;Melphalan;Cyclophosphamide;Thalidomide;Lenalidomide
7 A Randomized Phase III Trial of Melphalan and Dexamethasone (MDex) Versus Bortezomib, Melphalan and Dexamethasone (BMDex) for Untreated Patients With Systemic Light-Chain (AL) Amyloidosis Ineligible for Autologous Stem-Cell Transplantation Completed NCT01078454 Phase 3 melphalan;dexamethasone;bortezomib
8 Phase III Trial of Stem Cell Transplantation Compared to Parenteral Melphalan and Oral Dexamethasone in the Treatment of Primary Systemic Amyloidosis (AL) Completed NCT00477971 Phase 3 dexamethasone;melphalan
9 A Phase 3, Double-Blind, Multicenter Study to Evaluate the Efficacy and Safety of CAEL-101 and Plasma Cell Dyscrasia Treatment Versus Placebo and Plasma Cell Dyscrasia Treatment in Plasma Cell Dyscrasia Treatment Naïve Patients With Mayo Stage IIIb AL Amyloidosis Recruiting NCT04504825 Phase 3 CAEL-101;cyclophosphamide, bortezomib, and Dexamethasone (CyBorD) regimen
10 A Phase 3, Double-Blind, Multicenter Study to Evaluate the Efficacy and Safety of CAEL-101 and Plasma Cell Dyscrasia Treatment Versus Placebo and Plasma Cell Dyscrasia Treatment in Plasma Cell Dyscrasia Treatment Naïve Patients With Mayo Stage IIIa AL Amyloidosis Recruiting NCT04512235 Phase 3 CAEL-101;cyclophosphamide, bortezomib, and dexamethasone (CyBorD) regimen
11 A Phase 3, Randomized, Multicenter, Double-Blind, Placebo-Controlled, Efficacy and Safety Study of Birtamimab Plus Standard of Care vs. Placebo Plus Standard of Care in Mayo Stage IV Subjects With Light Chain (AL) Amyloidosis Recruiting NCT04973137 Phase 3 Birtamimab;Standard of Care Chemotherapy
12 A Randomized Phase II/III Trial of Doxycycline vs. Standard Supportive Therapy in Newly-diagnosed Cardiac AL Amyloidosis Patients Undergoing Bortezomib-based Therapy Active, not recruiting NCT03474458 Phase 2, Phase 3 Doxycycline;Standard of care therapy
13 A Randomized Phase 3 Study to Evaluate the Efficacy and Safety of Daratumumab in Combination With Cyclophosphamide, Bortezomib and Dexamethasone (CyBorD) Compared to CyBorD Alone in Newly Diagnosed Systemic AL Amyloidosis Active, not recruiting NCT03201965 Phase 3 Cyclophosphamide;Bortezomib;Dexamethasone, 40 mg;Daratumumab
14 An Open-label, Multi-center, Non-randomized Pivotal Phase 3 Study to Evaluate the Efficacy and Safety of [18F]Florbetaben Positron Emission Tomography (PET) Imaging to Diagnose Cardiac AL Amyloidosis Not yet recruiting NCT05184088 Phase 3 [18F]florbetaben
15 Phase III Trial of High-dose Melphalan and Stem Cell Transplantation Versus High-dose Melphalan and Bortezomib and Stem Cell Transplantation in Patients With AL Amyloidosis Terminated NCT02489500 Phase 3 Bortezomib;Melphalan;Neupogen
16 A Phase 3, Randomized, Multicenter, Double-Blind, Placebo-Controlled, 2-Arm, Efficacy and Safety Study of NEOD001 Plus Standard of Care Versus Placebo Plus Standard of Care in Subjects With Light Chain (AL) Amyloidosis Terminated NCT02312206 Phase 3 NEOD001
17 Randomized Phase II Trial of Two Stem Cell Doses To Reduce Transplant Induced Symptom Burden in High Risk Patients With Multiple Myeloma or Amyloidosis Completed NCT00651937 Phase 2 Melphalan;Granulocyte-colony stimulating factor (G-CSF)
18 A Phase 1/2, Open Label, Dose Escalation Study of Intravenous Administration of Single Agent NEOD001 in Subjects With Light Chain (AL) Amyloidosis Completed NCT01707264 Phase 1, Phase 2 NEOD001
19 A Prospective Single Center Trial of Treatment With Lenalidomide-Melphalan-Dexamethasone in Patients With AL Amyloidosis Completed NCT00883623 Phase 2 Lenalidomide
20 An Open-label, Phase II Study of Pomalidomide and Dexamethasone (PDex) for Previously Treated Patients With AL Amyloidosis. Completed NCT01510613 Phase 2 Pomalidomide and Dexamethasone
21 Phase II Trial of 4'-IODO-4'-Deoxydoxorubicin in Primary Amyloidosis (AL) Completed NCT00003853 Phase 2 4'-iodo-4'-deoxydoxorubicin
22 High Dose Chemotherapy and Autologous Stem Cell Rescue for Primary Amyloidosis Completed NCT00186407 Phase 2
23 Autologous Peripheral Blood Stem Cell Transplantation With High Dose Melphalan For Treatment Of Primary Amyloidosis (AL) Completed NCT00002810 Phase 2 melphalan
24 Doxycycline to Upgrade Organ Response in Light Chain (AL) Amyloidosis (DUAL) Trial: A Phase II Open Label Study of Oral Doxycycline Administered as an Adjunct to Plasma Cell Directed Therapy in Light Chain (AL) Amyloidosis Completed NCT02207556 Phase 2 Doxycycline
25 A Phase 2b, Randomized, Double-blind, Placebo-controlled Study of NEOD001 in Previously Treated Subjects With Light Chain (AL) Amyloidosis Who Have Persistent Cardiac Dysfunction Completed NCT02632786 Phase 2 NEOD001;Placebo
26 A Randomised Trial for the Treatment of Cardiac AMyloid Light-chain Amyloidosis With the Green Tea Compound Epigallocatechin-3-gallate (TAME-AL) Completed NCT02015312 Phase 2 Epigallocatechin-3-gallate (EGCG);Placebo
27 Phase 1/2 Study Of Ixazomib In Combination With Cyclophosphamide And Dexamethasone In Patients With Newly Diagnosed Immunoglobulin Light Chain AL Amyloidosis Completed NCT03236792 Phase 1, Phase 2 Ixazomib;Cyclophosphamide;Dexamethasone
28 A Phase I/II Trial of Pomalidomide and Dexamethasone in Subjects With Previously-Treated AL Amyloidosis Completed NCT01570387 Phase 1, Phase 2 Pomalidomide;Dexamethasone
29 A Phase I-II Trial of Daratumumab for the Treatment of Patients With AL Amyloidosis Completed NCT02841033 Phase 1, Phase 2 daratumumab
30 Phase II Study of the Combination of Bendamustine and Dexamethasone in Patients With Relapsed AL Amyloidosis Completed NCT01222260 Phase 2 Bendamustine;Dexamethasone
31 Phase II Trial of High-dose Melphalan and Bortezomib and Stem Cell Transplantation in Patients With AL Amyloidosis Completed NCT00790647 Phase 2 bortezomib;melphalan
32 Therapeutic Potential of Human Immune Globulin Intravenous (IGIV) in Patients With Cardiac-Associated Light Chain (AL) Amyloidosis Completed NCT00547365 Phase 1, Phase 2
33 Risk-Adapted Intravenous Melphalan With Stem Cell Transplant and Adjuvant Bortezomib and Dexamethasone for Recently Diagnosed Untreated Patients With Systemic Light-Chain (AL) Amyloidosis Completed NCT00458822 Phase 2 bortezomib;dexamethasone
34 A Phase II Trial of MRD (Melphalan, Lenalidomide and Dexamethasone) for Patients With AL Amyloidosis Completed NCT00679367 Phase 2 dexamethasone;lenalidomide;melphalan
35 A Phase II Trial of the Immunomodulatory Drug CC-5013 for Patients With AL Amyloidosis Completed NCT00091260 Phase 2 dexamethasone;lenalidomide
36 A Phase II Trial of Tandem Transplantation in AL Amyloidosis Completed NCT00075621 Phase 2 filgrastim;melphalan
37 Phase II Study of Dexamethasone/Alpha-Interferon in AL Amyloidosis Completed NCT00002849 Phase 2 dexamethasone
38 S0115, A Phase II Trial Evaluating Modified High Dose Melphalan (100 mg/m) And Autologous Peripheral Blood Stem Cell Supported Transplant (SCT) For High Risk Patients With Multiple Myeloma And/Or Light Chain Amyloidosis (AL Amyloidosis) (A BMT Study) Completed NCT00064337 Phase 2 cyclophosphamide;dexamethasone;melphalan;thalidomide
39 A Multicentre Open Label Phase II Study of Daratumumab in AL Amyloidosis Patients Not in VGPR or Better Completed NCT02816476 Phase 2 Daratumumab
40 A Single Arm Open Labeled Multicentre Phase 1b Dose Escalation Study of Carfilzomib Taken in Combination With Thalidomide and Dexamethasone in Relapsed AL Amyloidosis Completed NCT02545907 Phase 1, Phase 2 Carfilzomib;Thalidomide;Dexamethasone
41 Phase II Trial of Induction Therapy With Bortezomib and Dexamethasone Followed by High-Dose Melphalan and Stem Cell Transplantation in Patients With AL Amyloidosis Completed NCT01083316 Phase 2 Bortezomib;Dexamethasone;Melphalan
42 An Open-Label Phase 1/2 Study of VELCADE for Injection in Subjects With Light-Chain (AL)-Amyloidosis Completed NCT00298766 Phase 1, Phase 2 VELCADE
43 A Multicenter Phase I/II Dose Escalation Study of Lenalidomide in Combination With Melphalan and Dexamethasone in Subjects With Newly-diagnosed Light-chain (AL)-Amyloidosis Completed NCT00621400 Phase 1, Phase 2 Lenalidomide;Melphalan;Dexamethasone
44 An Open-label, Phase II Study of Cyclophosphamide, Lenalidomide and Dexamethasone (CLD) for Previously Treated Patients With AL Amyloidosis Completed NCT00607581 Phase 2 cyclophosphamide;lenalidomide;dexamethasone
45 Risk-adapted Therapy for AL Amyloidosis Completed NCT01527032 Phase 2 melphalan, thalidomide and dexamethasone
46 Single Autologous Transplant Followed by Consolidation and Maintenance for Participants ≥ 65 Years of Age Diagnosed With Multiple Myeloma or a Related Plasma Cell Malignancy Completed NCT01849783 Phase 2 dexamethasone;cisplatin;doxorubicin;cyclophosphamide;etoposide;bortezomib;thalidomide;melphalan
47 Phase II Study of Bortezomib, Cyclophosphamide and Dexamethasone in Patients With Primary Systemic Light Chain Amyloidosis Completed NCT01072773 Phase 2 bortezomib;cyclophosphamide;dexamethasone
48 Induction of Mixed Hematopoietic Chimerism in Patients Using Fludarabine, Low Dose TBI, PBSC Infusion and Post-Transplant Immunosuppression With Cyclosporine and Mycophenolate Mofetil Completed NCT00006251 Phase 1, Phase 2 fludarabine phosphate;cyclosporine;mycophenolate mofetil
49 A Multicentric, Phase II Trial of Lenalidomide, Cyclophosphamide and Dexamethasone in Patients With Primary Systemic Amyloidosis (AL) Newly Diagnosed, Not Candidates for Hematopoietic Stem Cell Transplantation Completed NCT01194791 Phase 2 Lenalidomide;Cyclophosphamide;Dexamethasone
50 Risk Adapted Intravenous Melphalan and Adjuvant Thalidomide and Dexamethasone for Untreated Patients With Primary Systemic Amyloidosis Completed NCT00089167 Phase 2 dexamethasone;melphalan;thalidomide

Search NIH Clinical Center for Immunoglobulin Light Chain Amyloidosis

Cochrane evidence based reviews: immunoglobulin light-chain amyloidosis

Genetic Tests for Immunoglobulin Light Chain Amyloidosis

Anatomical Context for Immunoglobulin Light Chain Amyloidosis

Organs/tissues related to Immunoglobulin Light Chain Amyloidosis:

MalaCards : Liver, Spleen, Kidney, Tongue, Heart, Bone, Salivary Gland

Publications for Immunoglobulin Light Chain Amyloidosis

Articles related to Immunoglobulin Light Chain Amyloidosis:

(show top 50) (show all 5041)
# Title Authors PMID Year
1
The molecular biology and clinical features of amyloid neuropathy. 53 62
17554795 2007
2
Current treatment in cardiac amyloidosis. 53 62
17078911 2006
3
[Case of primary amyloidosis complicated with intramuscular hemorrhage and Factor IX and X deficiencies]. 53 62
2061641 1991
4
Daratumumab in AL amyloidosis. 62
35507692 2022
5
The clinical features and outcomes of systemic light chain amyloidosis with hepatic involvement. 62
35481407 2022
6
Diagnostic amyloid light chain amyloidosis hospitalizations associated with high acuity and cost: analysis of the Premier Healthcare Database. 62
36268951 2022
7
Pilot Trial of Homebound Hematopoietic Cell Transplantation. 62
36182105 2022
8
Cardiovascular magnetic resonance in light-chain amyloidosis to guide treatment. 62
36239754 2022
9
Myocardial Uptake of 68Ga-Pentixafor in a Patient With Systemic Amyloidosis. 62
36342801 2022
10
Real-Life Evaluation of an Algorithm for the Diagnosis of Cardiac Amyloidosis. 62
36464537 2022
11
AL-Type Amyloidosis Involving Stomach and Lungs in 99m Tc-DPD Bone Scan. 62
35835133 2022
12
Feasibility of a Novel Academic BCMA-CART (HBI0101) for the Treatment of Relapsed and Refractory AL Amyloidosis. 62
36107221 2022
13
Plasma cell neoplasms and related entities-evolution in diagnosis and classification. 62
36414803 2022
14
Correction: First report of CART treatment in AL Amyloidosis and relapsed/refractory multiple myeloma. 62
36328380 2022
15
A multidisciplinary case report of multiple myeloma with renal and cardiac involvement: a look beyond amyloidosis. 62
36397026 2022
16
Isolated prostate amyloidosis; a case report. 62
36164377 2022
17
Modified High-Dose versus High-Dose Melphalan Conditioning in Older Patients Undergoing Autologous Stem Cell Transplantation for Immunoglobulin Light Chain Amyloidosis. 62
35970300 2022
18
Orbital amyloidosis and radiotherapy: A case report and review of literature. 62
35918273 2022
19
Primary amyloidosis of conjunctiva. 62
36467837 2022
20
Diving in the big blue: Atypical Mott cells in rapidly fatal primary amyloidosis. 62
36467785 2022
21
Suspecting and diagnosing transthyretin amyloid cardiomyopathy (ATTR-CM) in India: An Indian expert consensus. 62
36410415 2022
22
[Cardiac amyloidosis - important differential diagnosis in heart failure]. 62
36384153 2022
23
Leukocyte Chemotactic Factor 2 Amyloidosis (ALECT2) Distribution in a Mexican Population. 62
36370056 2022
24
Low QRS Voltages in Cardiac Amyloidosis: Clinical Correlates and Prognostic Value. 62
36444225 2022
25
Outcomes of heart transplantation in cardiac amyloidosis: an updated systematic review. 62
35595919 2022
26
Clinical features and outcomes of renal amyloidosis in Hong Kong. 62
36054582 2022
27
Macroglossia in rapidly progressive inclusion body myositis. 62
36349419 2022
28
Alcohol septal ablation for left ventricular outflow tract obstruction in cardiac amyloidosis: New indication for an established therapy. 62
36153647 2022
29
With M-protein positive, could transthyretin amyrodosis be easily excluded? Not necessarily! Wild-type transthyretin amyrodosis with Waldenström's macroglobulinaemia: a case report. 62
36339457 2022
30
Feasibility of 68Ga-Labeled Fibroblast Activation Protein Inhibitor PET/CT in Light-Chain Cardiac Amyloidosis. 62
36357138 2022
31
Clinical Outcomes and Effectiveness of Heart Transplantation in Patients With Systemic Light-chain Cardiac Amyloidosis. 62
35706094 2022
32
Advances in the treatment of light chain amyloidosis. 62
35943427 2022
33
Concurrent light chain amyloidosis and proximal tubulopathy: Insights into different aggregation behavior-A case report. 62
36467828 2022
34
Systemic light chain amyloidosis myopathy responsive to daratumumab monotherapy. 62
36403110 2022
35
Outcomes by Cardiac Stage in Patients With Newly Diagnosed AL Amyloidosis: Phase 3 ANDROMEDA Trial. 62
36444227 2022
36
[Interest of daratumumab in refractory AL amyloidosis in a 96-year-old patient]. 62
35934598 2022
37
Early cardiac response is possible in stage IIIb cardiac AL amyloidosis and is associated with prolonged survival. 62
35772003 2022
38
Understanding real-world treatment patterns and clinical outcomes in AL amyloidosis patients diagnosed in Canada: A population-based cohort study. 62
36467790 2022
39
AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: JACC: CardioOncology State-of-the-Art Review. 62
36444232 2022
40
Role of Daratumumab in Cardiac AL Amyloidosis. 62
36444231 2022
41
A rare case of isolated laryngotracheal amyloidosis with airway narrowing and vocal fold involvement. 62
36065250 2022
42
Treatment of Localized Amyloid Light Chain Amyloidosis With External Beam Radiation Therapy. 62
36088238 2022
43
Validation of an administrative coding algorithm for the identification of individuals with amyloid light chain amyloidosis using administrative data. 62
36409003 2022
44
Graded Cardiac Response Criteria for Patients With Systemic Light Chain Amyloidosis. 62
36215675 2022
45
Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal. 62
36358666 2022
46
Treatment of amyloid light chain cardiac amyloidosis: systematic review and future directions. 62
36206073 2022
47
Truncal sensory polyneuropathy in light-chain amyloidosis. 62
36288916 2022
48
Unusual purpura as a first sign of amyloid light chain amyloidosis. 62
36174634 2022
49
Violaceous truncal plaques consistent with amyloid light-chain amyloidosis. 62
34263931 2022
50
A prospective, randomized trial of patient-reported outcome measures to drive management decisions in hematology and oncology. 62
35928285 2022

Variations for Immunoglobulin Light Chain Amyloidosis

Expression for Immunoglobulin Light Chain Amyloidosis

Search GEO for disease gene expression data for Immunoglobulin Light Chain Amyloidosis.

Pathways for Immunoglobulin Light Chain Amyloidosis

GO Terms for Immunoglobulin Light Chain Amyloidosis

Cellular components related to Immunoglobulin Light Chain Amyloidosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 10.4 TTR NPPB LYZ LECT2 GSN F9
2 extracellular exosome GO:0070062 10.23 ALB APCS APOA1 B2M CD38 F9
3 extracellular region GO:0005576 10.02 ALB APCS APOA1 B2M F9 GSN
4 blood microparticle GO:0072562 9.92 ALB APCS APOA1 GSN
5 cardiac myofibril GO:0097512 9.67 TNNT2 TNNI3
6 cardiac Troponin complex GO:1990584 9.56 TNNT2 TNNI3
7 troponin complex GO:0005861 9.1 TNNT2 TNNT1 TNNI3

Biological processes related to Immunoglobulin Light Chain Amyloidosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 retina homeostasis GO:0001895 9.8 LYZ B2M ALB
2 sarcomere organization GO:0045214 9.73 TNNT2 TNNT1 MYOM2
3 muscle contraction GO:0006936 9.7 TNNT2 TNNT1 TNNI3 MYOM2
4 regulation of system process GO:0044057 8.96 TNNT2 TNNT1
5 regulation of muscle contraction GO:0006937 8.8 TNNT2 TNNT1 TNNI3

Molecular functions related to Immunoglobulin Light Chain Amyloidosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 10.13 TTR TNNT2 SDC1 LYZ LECT2 CD38
2 troponin T binding GO:0031014 9.26 TNNT1 TNNI3
3 troponin C binding GO:0030172 8.92 TNNT2 TNNI3

Sources for Immunoglobulin Light Chain Amyloidosis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
Content
Loading form....