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MCID: ALM001
MIFTS: 50

Al Amyloidosis

Categories: Blood diseases, Bone diseases, Genetic diseases, Immune diseases, Metabolic diseases, Nephrological diseases, Neuronal diseases, Rare diseases
Genes Tissues Related diseases Publications Symptoms & Phenotypes Drugs
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Aliases & Classifications for Al Amyloidosis

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MalaCards integrated aliases for Al Amyloidosis:

Name: Al Amyloidosis 52
Primary Systemic Amyloidosis 52 71
Primary Amyloidosis 52 71
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 Deposition Disease 71
Primary Systemic Al Amyloidosis 52
Amyloidosis Primary Systemic 52
Light Chain Amyloidosis 52
Primary Al Amyloidosis 52
Systemic Al Amyloidsis 52
Lysozyme Amyloidosis 58
Alys Amyloidosis 58
Amyloidosis Al 52

Characteristics:

Orphanet epidemiological data:

58
alys amyloidosis
Inheritance: Autosomal dominant; Prevalence: <1/1000000 (Worldwide);

Classifications:

MalaCards categories:
Global: Rare diseases Genetic diseases Metabolic diseases
Anatomical: Nephrological diseases Bone diseases Immune diseases Neuronal diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 33
Orphanet: 58  
Rare renal diseases
Rare systemic and rhumatological diseases


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Summaries for Al Amyloidosis

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NIH Rare Diseases : 52 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. Treatment may include chemotherapy directed at the abnormal plasma cells , stem cell transplantation , or other treatments based on which symptoms have developed.

MalaCards based summary : Al Amyloidosis, also known as primary systemic amyloidosis, is related to acute kidney failure and hereditary amyloidosis. An important gene associated with Al Amyloidosis is LYZ (Lysozyme). The drugs Ketamine and Clotrimazole have been mentioned in the context of this disorder. Affiliated tissues include heart, bone and kidney, and related phenotypes are fatigue and hypertrophic cardiomyopathy

Wikipedia : 74 Amyloid light-chain (AL) amyloidosis, also known as primary amyloidosis, is the most common form of... more...

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Related Diseases for Al Amyloidosis

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Diseases in the Amyloidosis family:

Al Amyloidosis Amyloidosis Aa
Hereditary Amyloidosis Primary Localized Amyloidosis
Ah Amyloidosis

Diseases related to Al Amyloidosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 414)
# Related Disease Score Top Affiliating Genes
1 acute kidney failure 30.0 B2M ADAMTS13
2 hereditary amyloidosis 30.0 LYZ B2M
3 intermittent claudication 29.7 VWF B2M
4 purpura 29.7 VWF ADAMTS13
5 amyloidosis, familial visceral 29.6 LYZ B2M
6 acquired von willebrand syndrome 29.5 VWF ADAMTS13
7 disseminated intravascular coagulation 29.4 VWF ADAMTS13
8 von willebrand's disease 29.2 VWF ADAMTS13
9 hydronephrosis 29.2 VWF B2M
10 hemorrhagic disease 29.2 VWF ADAMTS13
11 portal hypertension 29.1 VWF ADAMTS13
12 hemangioma 28.7 VWF ACTC1
13 myeloma, multiple 11.9
14 primary localized amyloidosis 11.7
15 light chain deposition disease 11.4
16 amyloidosis 11.3
17 amyloidosis, primary localized cutaneous, 1 11.2
18 amyloidosis, primary localized cutaneous, 2 11.2
19 amyloidosis, primary localized cutaneous, 3 11.2
20 amyloidosis, hereditary, transthyretin-related 11.1
21 plasma cell neoplasm 10.7
22 nephrotic syndrome 10.6
23 congestive heart failure 10.4
24 amyloidosis aa 10.4
25 macroglossia 10.4
26 factor x deficiency 10.4
27 monoclonal gammopathy of uncertain significance 10.3
28 neuropathy 10.3
29 end stage renal disease 10.3
30 macroglobulinemia 10.3
31 kidney disease 10.3
32 polyneuropathy 10.3
33 myopathy 10.3
34 peripheral nervous system disease 10.3
35 carpal tunnel syndrome 10.3
36 pure autonomic failure 10.3
37 cholestasis 10.2
38 plasmacytoma 10.2
39 hypertrophic cardiomyopathy 10.2
40 restrictive cardiomyopathy 10.2
41 diarrhea 10.2
42 autonomic neuropathy 10.1
43 constipation 10.1
44 interstitial nephritis 10.1
45 lymphoplasmacytic lymphoma 10.1
46 colitis 10.1
47 intestinal pseudo-obstruction 10.1
48 glomerulonephritis 10.1
49 systemic scleroderma 10.1
50 atrial standstill 1 10.0

Graphical network of the top 20 diseases related to Al Amyloidosis:



Diseases related to Al Amyloidosis
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Symptoms & Phenotypes for Al Amyloidosis

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Human phenotypes related to Al Amyloidosis:

31 (show top 50) (show all 53)
# Description HPO Frequency HPO Source Accession
1 fatigue 31 hallmark (90%) HP:0012378
2 hypertrophic cardiomyopathy 31 hallmark (90%) HP:0001639
3 hepatomegaly 31 frequent (33%) HP:0002240
4 proteinuria 31 frequent (33%) HP:0000093
5 nephropathy 31 frequent (33%) HP:0000112
6 renal insufficiency 31 frequent (33%) HP:0000083
7 dyspnea 31 frequent (33%) HP:0002094
8 arthralgia 31 frequent (33%) HP:0002829
9 congestive heart failure 31 frequent (33%) HP:0001635
10 nephrotic syndrome 31 frequent (33%) HP:0000100
11 enlarged kidney 31 frequent (33%) HP:0000105
12 lymphadenopathy 31 frequent (33%) HP:0002716
13 pedal edema 31 frequent (33%) HP:0010741
14 abnormality of chromosome segregation 31 frequent (33%) HP:0002916
15 interstitial pulmonary abnormality 31 frequent (33%) HP:0006530
16 pulmonary edema 31 frequent (33%) HP:0100598
17 multiple myeloma 31 frequent (33%) HP:0006775
18 monoclonal immunoglobulin m proteinemia 31 frequent (33%) HP:0005508
19 arthropathy 31 frequent (33%) HP:0003040
20 abnormal ekg 31 frequent (33%) HP:0003115
21 elevated alkaline phosphatase 31 frequent (33%) HP:0003155
22 chronic constipation 31 frequent (33%) HP:0012450
23 acute infectious pneumonia 31 frequent (33%) HP:0011949
24 macroglossia 31 occasional (7.5%) HP:0000158
25 malabsorption 31 occasional (7.5%) HP:0002024
26 abnormal blistering of the skin 31 occasional (7.5%) HP:0008066
27 papule 31 occasional (7.5%) HP:0200034
28 hepatitis 31 occasional (7.5%) HP:0012115
29 xerostomia 31 occasional (7.5%) HP:0000217
30 hematuria 31 occasional (7.5%) HP:0000790
31 gastrointestinal hemorrhage 31 occasional (7.5%) HP:0002239
32 hypertension 31 occasional (7.5%) HP:0000822
33 purpura 31 occasional (7.5%) HP:0000979
34 osteoarthritis 31 occasional (7.5%) HP:0002758
35 goiter 31 occasional (7.5%) HP:0000853
36 keratoconjunctivitis sicca 31 occasional (7.5%) HP:0001097
37 abnormality of bone marrow cell morphology 31 occasional (7.5%) HP:0005561
38 asplenia 31 occasional (7.5%) HP:0001746
39 pleural effusion 31 occasional (7.5%) HP:0002202
40 upper airway obstruction 31 occasional (7.5%) HP:0002781
41 polyneuropathy 31 occasional (7.5%) HP:0001271
42 aortic root aneurysm 31 occasional (7.5%) HP:0002616
43 orthostatic hypotension due to autonomic dysfunction 31 occasional (7.5%) HP:0004926
44 adrenal insufficiency 31 occasional (7.5%) HP:0000846
45 skin nodule 31 occasional (7.5%) HP:0200036
46 intermittent claudication 31 occasional (7.5%) HP:0004417
47 bradycardia 31 occasional (7.5%) HP:0001662
48 autonomic bladder dysfunction 31 occasional (7.5%) HP:0005341
49 abnormality of the submandibular glands 31 occasional (7.5%) HP:0010287
50 mechanical ileus 31 occasional (7.5%) HP:0010676

MGI Mouse Phenotypes related to Al Amyloidosis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.02 ACTC1 ADAMTS13 B2M LYZ VWF
Sources

Drugs & Therapeutics for Al Amyloidosis

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Drugs for Al Amyloidosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 225)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ketamine Approved, Vet_approved Phase 3 6740-88-1 3821
2
Clotrimazole Approved, Vet_approved Phase 3 23593-75-1 2812
3
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
4
Ondansetron Approved Phase 3 99614-02-5 4595
5
Dalteparin Approved Phase 3 9005-49-6
6
Tinzaparin Approved Phase 3 9041-08-1, 9005-49-6 25244225
7
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
8
Darbepoetin alfa Approved, Investigational Phase 3 11096-26-7, 209810-58-2
9
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
10
Iron Approved, Experimental Phase 3 15438-31-0, 7439-89-6 27284 23925
11
Caspofungin Approved Phase 3 162808-62-0, 179463-17-3 2826718 468682
12
Amphotericin B Approved, Investigational Phase 3 1397-89-3 5280965 14956
13
Doxycycline Approved, Investigational, Vet_approved Phase 2, Phase 3 564-25-0 54671203
14
Lenalidomide Approved Phase 3 191732-72-6 216326
15
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
16
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
17
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
18
Bortezomib Approved, Investigational Phase 3 179324-69-7 387447 93860
19
Daratumumab Approved Phase 3 945721-28-8
20
Ixazomib Approved, Investigational Phase 3 1072833-77-2
21
Lenograstim Approved, Investigational Phase 3 135968-09-1
22
Melphalan Approved Phase 3 148-82-3 460612 4053
23
Sargramostim Approved, Investigational Phase 3 83869-56-1, 123774-72-1
24
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
25
Ginseng Approved, Investigational, Nutraceutical Phase 3 50647-08-0
26 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
27 Molgramostim Investigational Phase 3 99283-10-0
28 Anesthetics, Dissociative Phase 3
29 Hormones Phase 3
30 Anti-Infective Agents Phase 3
31 Anti-Bacterial Agents Phase 3
32 Antiparasitic Agents Phase 3
33 Antiprotozoal Agents Phase 3
34 Dermatologic Agents Phase 3
35 Neurotransmitter Agents Phase 3
36 Analgesics Phase 3
37 Liver Extracts Phase 3
38 Antifungal Agents Phase 3
39 Anti-Anxiety Agents Phase 3
40 Psychotropic Drugs Phase 3
41 Antipsychotic Agents Phase 3
42 Fibrinolytic Agents Phase 3
43 Heparin, Low-Molecular-Weight Phase 3
44 Excitatory Amino Acid Antagonists Phase 3
45 Narcotics Phase 3
46 Analgesics, Opioid Phase 3
47 Cola Phase 3
48 Hematinics Phase 3
49 Epoetin alfa Phase 3 113427-24-0
50 Anesthetics Phase 3

Interventional clinical trials:

(show top 50) (show all 192)
# Name Status NCT ID Phase Drugs
1 A Pilot Study to Evaluate 18F Florbetapir Binding to Cardiac Amyloid in Patients Undergoing Chemotherapy Not yet recruiting NCT03333551 Phase 4 F18 Florbetapir (amyvid) cardiac PET/CT imaging
2 A Randomized Double-Blind Controlled Trial of Ketamine Versus Placebo in Conjunction With Best Pain Management in Neuropathic Pain in Cancer Patients Unknown status NCT01316744 Phase 3 ketamine hydrochloride
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 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
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 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
7 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
8 Stem Cell Transplant as Standard Therapy for Symptomatic Multiple Myeloma Completed NCT00004165 Phase 3 melphalan
9 Phase III, Randomized, Double-Blind, Placebo-Controlled Crossover Trial of Ondansetron in the Control of Chronic Nausea and Vomiting Not Due to Antineoplastic Therapy in Patients With Advanced Cancer Completed NCT00006348 Phase 3 ondansetron
10 A Phase III Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Effects of Fragmin (5,000 IU Subcutaneously) in Preventing Catheter-Related Complications When Given Daily to Cancer Patients With Central Venous Catheters Completed NCT00006083 Phase 3 Fragmin
11 A Phase III Double-Blind Equivalence Study of Two Different Formulations of Slow-Release Morphine Followed by a Randomization Between Dextromethorphan or Placebo Plus Statex SR for Chronic Cancer Pain Relief in Terminally Ill Patients Completed NCT00003687 Phase 3 dextromethorphan hydrobromide;morphine sulfate
12 A Randomized, Open-Label, Multicenter Study Of Darbepoetin Alfa Administered Once Every Two Weeks (Q2W) Compared With rHuEPO Administered Once Every Week (QW) For The Treatment Of Anemia In Subjects With Non-Myeloid Malignancies Receiving Multiple Chemotherapy Completed NCT00070382 Phase 3 darbepoetin alfa;epoetin alfa
13 Does Hypericum Reduce Fatigue in Cancer Patients on Chemotherapy? A Randomized, Double-Blind, Placebo-Controlled Clinical Trial Completed NCT00005805 Phase 3
14 A Phase III, Randomized Study of the Effects of Parenteral Iron, Oral Iron, or No Iron Supplementation on the Erythropoietic Response to Darbepoetin Alfa for Cancer Patients With Chemotherapy-Associated Anemia Completed NCT00661999 Phase 3 sodium ferric gluconate complex in sucrose
15 Preparatory Aid to Improve Decision Making About Cancer Clinical Trials (PRE-ACT) Completed NCT00750009 Phase 3
16 A Strategic Study to Determine the Optimal Moment to Initiate Systemic Antifungal Therapy With Ambisome in Granulocytopenic Cancer Patients With Unexplained Fever Refractory to Empirical Antibacterials Completed NCT00003938 Phase 3 liposomal amphotericin B
17 A Multicenter, Double-Blind, Randomized, Comparative Study To Evaluate The Safety, Tolerability, And Efficacy Of MK-0991 Versus (Amphotericin B) Liposome For Injection As Empirical Therapy In Patients With Persistent Fever And Neutropenia Completed NCT00008359 Phase 3 caspofungin acetate;liposomal amphotericin B
18 The Use of American Ginseng (Panax Quinquefolius) to Improve Cancer-Related Fatigue: A Randomized, Double-Blind, Placebo-Controlled Phase III Study Completed NCT00719563 Phase 3 American ginseng
19 Phase III Randomized Trial of an Opioid Titration Order Sheet Compared to Standard of Care in Patients With Cancer Related Pain. Completed NCT00666211 Phase 3
20 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
21 Frontline Lenalidomide for AL Amyloidosis Involving Myocardium: Investigation of Organ Reversing Capacity of Lenalidomide Recruiting NCT04298372 Phase 3 Lenalidomide 25mg
22 A Randomized Phase II/III Trial of Doxycycline vs. Standard Supportive Therapy in Newly-diagnosed Cardiac AL Amyloidosis Patients Undergoing Bortezomib-based Therapy Recruiting NCT03474458 Phase 2, Phase 3 Doxycycline;Standard of care therapy
23 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 Active, not recruiting NCT01659658 Phase 3 IXAZOMIB;Dexamethasone;Melphalan;Cyclophosphamide;Thalidomide;Lenalidomide
24 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
25 Assessment of 18F-Florbetaben Whole-body PET Imaging for the Comprehensive Detection of Cardiac and Extracardiac Sites of Amyloid Deposits Not yet recruiting NCT03616496 Phase 3 Neuraceq 300MBq/mL Solution for Injection for PET imaging
26 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
27 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
28 A Phase I/II Trial of Lenalidomide Combined With Cyclophosphamide and Intermediate Dose Dexamethasone in Patients With Primary (AL) Systemic Amyloidosis Unknown status NCT00981708 Phase 1, Phase 2 Lenalidomide, Dexamethasone and Cyclophosphamide
29 A Single Arm Open Labeled Multicentre Phase 1b Dose Escalation Study of Carfilzomib Taken in Combination With Thalidomide and Dexamethasone in Relapsed AL Amyloidosis Unknown status NCT02545907 Phase 1, Phase 2 Carfilzomib;Thalidomide;Dexamethasone
30 Myeloblative Therapy With Autologous Hematopoietic Stem Cell Transplantation in Patients With Multiple Myeloma and B-Cell Malignancies Unknown status NCT00003163 Phase 2 cyclophosphamide;melphalan
31 A Phase II Trial of the Immunomodulatory Drug CC-5013 for Patients With AL Amyloidosis Completed NCT00091260 Phase 2 dexamethasone;lenalidomide
32 Risk Adapted Intravenous Melphalan and Adjuvant Thalidomide and Dexamethasone for Untreated Patients With Primary Systemic Amyloidosis Completed NCT00089167 Phase 2 dexamethasone;melphalan;thalidomide
33 Phase II Trial of 4'-IODO-4'-Deoxydoxorubicin in Primary Amyloidosis (AL) Completed NCT00003853 Phase 2 4'-iodo-4'-deoxydoxorubicin
34 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
35 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
36 Phase II Study of Dexamethasone/Alpha-Interferon in AL Amyloidosis Completed NCT00002849 Phase 2 dexamethasone
37 A Phase II Trial of MRD (Melphalan, Lenalidomide and Dexamethasone) for Patients With AL Amyloidosis Completed NCT00679367 Phase 2 dexamethasone;lenalidomide;melphalan
38 Phase II Trial of High-dose Melphalan and Bortezomib and Stem Cell Transplantation in Patients With AL Amyloidosis Completed NCT00790647 Phase 2 bortezomib;melphalan
39 A Prospective Single Center Trial of Treatment With Lenalidomide-Melphalan-Dexamethasone in Patients With AL Amyloidosis Completed NCT00883623 Phase 2 Lenalidomide
40 A Phase II Study of High-Dose Melphalan With Hematopoietic Stem Cell Reconstitution for Patients With Primary Systemic Amyloidosis Completed NCT00003353 Phase 2 melphalan
41 Therapeutic Potential of Human Immune Globulin Intravenous (IGIV) in Patients With Cardiac-Associated Light Chain (AL) Amyloidosis Completed NCT00547365 Phase 1, Phase 2
42 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
43 A Phase II Trial of Lenalidomide (Revlimid®), Cyclophosphamide and Dexamethasone in Patients With Primary Systemic Amyloidosis Completed NCT00564889 Phase 2 cyclophosphamide;dexamethasone;lenalidomide
44 Phase II Study of the Combination of Bendamustine and Dexamethasone in Patients With Relapsed AL Amyloidosis Completed NCT01222260 Phase 2 Bendamustine;Dexamethasone
45 A Phase II Trial of CC-5013 in Patients With Primary Systemic Amyloidosis Completed NCT00166413 Phase 2 CC-5013
46 High Dose Chemotherapy and Autologous Stem Cell Rescue for Primary Amyloidosis Completed NCT00186407 Phase 2
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 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
49 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
50 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

Search NIH Clinical Center for Al Amyloidosis

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Genetic Tests for Al Amyloidosis

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Anatomical Context for Al Amyloidosis

About this section

MalaCards organs/tissues related to Al Amyloidosis:

40
Heart, Bone, Kidney, Liver, Bone Marrow, Skin, Testes
Sources

Publications for Al Amyloidosis

About this section

Articles related to Al Amyloidosis:

(show top 50) (show all 2434)
# Title Authors PMID Year
1
Primary Localized Conjunctival Amyloidosis Presenting Corneal Whorl-like Opacity Patterns. 61
32301810 2020
2
Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment. 61
32267020 2020
3
Clinicopathologic Assessment of Monoclonal Immunoglobulin-associated Renal Disease in the Kidney Allograft: A Retrospective Study and Review of the Literature. 61
31634325 2020
4
Quantitative [18F]florbetapir PET/CT may identify lung involvement in patients with systemic AL amyloidosis. 61
31807884 2020
5
Amyloid purpura of the breasts in a patient with systemic AL amyloidosis. 61
32200565 2020
6
Nonchemotherapy Treatment of Immunoglobulin Light Chain Amyloidosis. 61
32526750 2020
7
Challenges in the Management of patients with systemic light chain (AL) amyloidosis during the COVID-19 pandemic. 61
32480420 2020
8
A novel risk score to predict survival in advanced heart failure due to cardiac amyloidosis. 61
31630214 2020
9
CD38 and Anti-CD38 Monoclonal Antibodies in AL Amyloidosis: Targeting Plasma Cells and beyond. 61
32531894 2020
10
Acquired and inherited amyloidosis: Knowledge driving patients' care. 61
32378274 2020
11
Solid Organ Transplantation in Amyloidosis. 61
32535598 2020
12
Outcomes in patients with cardiac amyloidosis and implantable cardioverter-defibrillator. 61
32514579 2020
13
Presence of t(11;14) in AL amyloidosis as a marker of response when treated with a bortezomib-based regimen. 61
32551974 2020
14
A retrospective analysis of treatment outcomes in 45 patients with cardiac light-chain amyloidosis: a single-center experience in Japan. 61
32020505 2020
15
Involvement of small nerve fibres and autonomic nervous system in AL amyloidosis: comprehensive characteristics and clinical implications. 61
31971444 2020
16
Cyclophosphamide, Bortezomib and Dexamethasone (CyBorD) for the Treatment of Newly Diagnosed AL Amyloidosis: Impact of Response on Survival Outcomes. 61
32146104 2020
17
A real-life cohort study of immunoglobulin light-chain (AL) amyloidosis patients ineligible for autologous stem cell transplantation due to severe cardiac involvement or advanced disease. 61
32052655 2020
18
The use of PROMIS patient-reported outcomes (PROs) to inform light chain (AL) amyloid disease severity at diagnosis. 61
31971467 2020
19
Adjuvant doxycycline to enhance anti-amyloid effects: Results from the dual phase 2 trial. 61
32529175 2020
20
Carfilzomib-induced hemolysis is noticeably common but rarely shows features of thrombotic microangiopathy: A retrospective study. 61
32115785 2020
21
Patient Outcomes in Light Chain (AL) amyloidosis: the Clock is Ticking from Symptoms to Diagnosis. 61
32564450 2020
22
The value of screening biopsies in light-chain (AL) and transthyretin (ATTR) amyloidosis. 61
32495369 2020
23
Diagnostic Accuracy of [11C]PIB Positron Emission Tomography for Detection of Cardiac Amyloidosis. 61
32417330 2020
24
Improved Quantification of Cardiac Amyloid Burden in Systemic Light Chain Amyloidosis: Redefining Early Disease? 61
32417333 2020
25
Outcomes with early vs. deferred stem cell transplantation in light chain amyloidosis. 61
32518290 2020
26
Prognosis and Staging of AL Amyloidosis. 61
32570242 2020
27
Diagnosis and management of systemic light chain AL amyloidosis. 61
32562825 2020
28
Updates in the Diagnosis and Management of AL Amyloidosis. 61
32394186 2020
29
Safety and Tolerability of Neurohormonal Antagonism in Cardiac Amyloidosis. 61
32475765 2020
30
Amyloid cardiomyopathy. 61
32107564 2020
31
Use of ixazomib, lenalidomide and dexamethasone in patients with relapsed amyloid light-chain amyloidosis. 61
31984481 2020
32
Recurrent pericardial effusion with pericardial amyloid deposition: a case report and literature review. 61
31927216 2020
33
Budd-Chiari Syndrome: An Unusual Complication of AL Amyloidosis 61
32126743 2020
34
Massive Hepatomegaly Secondary to Amyloidosis with Normal Liver Chemistries. 61
32518538 2020
35
Pomalidomide and dexamethasone grant rapid haematologic responses in patients with relapsed and refractory AL amyloidosis: a European retrospective series of 153 patients. 61
32449385 2020
36
Outcome of patients with severe AL amyloidosis and biopsy-proven renal involvement ineligible for bone marrow transplantation. 61
32472525 2020
37
The impact and importance of achieving a complete haematological response prior to renal transplantation in AL amyloidosis. 61
32444621 2020
38
Rapid response to single agent daratumumab is associated with improved progression-free survival in relapsed/refractory AL amyloidosis. 61
32408823 2020
39
Prognostic Role of Beta-2 Microglobulin in Patients with Light Chain Amyloidosis Treated with Autologous Stem Cell Transplantation. 61
32422250 2020
40
A Case of Primary Systemic Amyloidosis Involving the Sinonasal Tract. 61
32364447 2020
41
Venetoclax for the treatment of translocation (11;14) AL amyloidosis. 61
32393733 2020
42
Diagnostic score of cardiac involvement in AL amyloidosis. 61
31292624 2020
43
A powerful oral triplet for AL amyloidosis. 61
32011727 2020
44
IgM AL amyloidosis: delineating disease biology and outcomes with clinical, genomic and bone marrow morphological features. 61
31780812 2020
45
Impact of minimal residual negativity using next generation flow cytometry on outcomes in light chain amyloidosis. 61
32010993 2020
46
Light chain amyloidosis induced inflammatory changes in cardiomyocytes and adipose-derived mesenchymal stromal cells. 61
31796914 2020
47
Evaluation of Cardiac AL Amyloidosis: More Than Amyloid Deposit in the Heart. 61
32381171 2020
48
Reply: Evaluation of Cardiac AL Amyloidosis: More Than Amyloid Deposit in the Heart. 61
32381172 2020
49
Bendamustine With Dexamethasone in Relapsed/Refractory Systemic Light-Chain Amyloidosis: Results of a Phase II Study. 61
32083996 2020
50
Outcomes following cardioversion for patients with cardiac amyloidosis and atrial fibrillation or atrial flutter. 61
32004797 2020
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Variations for Al Amyloidosis

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Expression for Al Amyloidosis

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Search GEO for disease gene expression data for Al Amyloidosis.
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Pathways for Al Amyloidosis

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GO Terms for Al Amyloidosis

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Cellular components related to Al Amyloidosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.62 VWF LYZ B2M ADAMTS13
2 extracellular exosome GO:0070062 9.56 VWF LYZ B2M ACTC1
3 extracellular space GO:0005615 9.46 LYZ B2M ADAMTS13 ACTC1
4 specific granule lumen GO:0035580 8.96 LYZ B2M
5 tertiary granule lumen GO:1904724 8.62 LYZ B2M

Biological processes related to Al Amyloidosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 platelet activation GO:0030168 9.16 VWF ADAMTS13
2 hemostasis GO:0007599 8.96 VWF ADAMTS13
3 retina homeostasis GO:0001895 8.62 LYZ B2M

Molecular functions related to Al Amyloidosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 integrin binding GO:0005178 8.62 VWF ADAMTS13
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Sources for Al Amyloidosis

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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