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MCID: ALM001
MIFTS: 54

Al Amyloidosis

Categories: Blood diseases, Bone diseases, Genetic diseases, Immune diseases, Metabolic diseases, Muscle diseases, Nephrological diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Al Amyloidosis

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MalaCards integrated aliases for Al Amyloidosis:

Name: Al Amyloidosis 20
Primary Systemic Amyloidosis 20 71
Primary Amyloidosis 20 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 20
Amyloidosis Primary Systemic 20
Light Chain Amyloidosis 20
Primary Al Amyloidosis 20
Systemic Al Amyloidsis 20
Lysozyme Amyloidosis 58
Amyloidosis Primary 54
Alys Amyloidosis 58
Amyloidosis Al 20

Characteristics:

Orphanet epidemiological data:

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

Classifications:

MalaCards categories:
Global: Rare diseases Metabolic diseases Genetic diseases
Anatomical: Nephrological diseases Bone diseases Neuronal diseases Blood diseases Immune diseases Muscle 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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GARD : 20 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 amyloidosis, hereditary, transthyretin-related and amyloidosis. An important gene associated with Al Amyloidosis is LYZ (Lysozyme), and among its related pathways/superpathways are Innate Immune System and Antigen Presentation- Folding, assembly and peptide loading of class I MHC. The drugs Ketamine and Clotrimazole have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, bone and tongue, 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 403)
# Related Disease Score Top Affiliating Genes
1 amyloidosis, hereditary, transthyretin-related 31.4 TTR B2M
2 amyloidosis 31.4 TTR LYZ B2M
3 amyloidosis aa 30.7 TTR B2M
4 carpal tunnel syndrome 30.5 TTR B2M
5 amyloid tumor 30.0 TTR B2M
6 restrictive cardiomyopathy 30.0 TTR ACTC1
7 acquired von willebrand syndrome 29.9 VWF CALR
8 hydronephrosis 29.8 VWF B2M
9 sjogren syndrome 29.7 CALR B2M
10 hypertrophic cardiomyopathy 29.6 VWF TTR ACTC1
11 hereditary amyloidosis 29.3 TTR LYZ B2M
12 amyloidosis, familial visceral 29.2 TTR LYZ B2M
13 dilated cardiomyopathy 29.1 VWF CALR B2M ACTC1
14 myeloma, multiple 11.4
15 primary localized amyloidosis 11.2
16 primary cutaneous amyloidosis 11.2
17 lichen amyloidosis 11.0
18 light chain deposition disease 11.0
19 amyloidosis, primary localized cutaneous, 1 11.0
20 amyloidosis, primary localized cutaneous, 2 11.0
21 amyloidosis, primary localized cutaneous, 3 11.0
22 plasma cell neoplasm 10.7
23 nephrotic syndrome 10.7
24 proteinuria, chronic benign 10.6
25 macroglossia 10.4
26 neuropathy 10.4
27 factor x deficiency 10.4
28 monoclonal gammopathy of uncertain significance 10.4
29 polyneuropathy 10.3
30 peripheral nervous system disease 10.3
31 macroglobulinemia 10.3
32 myopathy 10.3
33 end stage renal disease 10.3
34 pure autonomic failure 10.3
35 cholestasis 10.3
36 acute kidney failure 10.3
37 plasmacytoma 10.3
38 kidney disease 10.2
39 interstitial nephritis 10.2
40 engraftment syndrome 10.2
41 atrial standstill 1 10.2
42 autonomic neuropathy 10.2
43 constipation 10.2
44 purpura 10.2
45 congestive heart failure 10.2
46 lymphoplasmacytic lymphoma 10.1
47 colitis 10.1
48 intestinal pseudo-obstruction 10.1
49 thrombocytopenia 10.1
50 glomerulonephritis 10.1

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 congestive heart failure 31 frequent (33%) HP:0001635
8 nephrotic syndrome 31 frequent (33%) HP:0000100
9 dyspnea 31 frequent (33%) HP:0002094
10 arthralgia 31 frequent (33%) HP:0002829
11 enlarged kidney 31 frequent (33%) HP:0000105
12 abnormality of chromosome segregation 31 frequent (33%) HP:0002916
13 lymphadenopathy 31 frequent (33%) HP:0002716
14 pedal edema 31 frequent (33%) HP:0010741
15 pulmonary edema 31 frequent (33%) HP:0100598
16 multiple myeloma 31 frequent (33%) HP:0006775
17 monoclonal immunoglobulin m proteinemia 31 frequent (33%) HP:0005508
18 arthropathy 31 frequent (33%) HP:0003040
19 elevated alkaline phosphatase 31 frequent (33%) HP:0003155
20 abnormal ekg 31 frequent (33%) HP:0003115
21 chronic constipation 31 frequent (33%) HP:0012450
22 acute infectious pneumonia 31 frequent (33%) HP:0011949
23 abnormal pulmonary interstitial morphology 31 frequent (33%) HP:0006530
24 hypertension 31 occasional (7.5%) HP:0000822
25 macroglossia 31 occasional (7.5%) HP:0000158
26 malabsorption 31 occasional (7.5%) HP:0002024
27 hepatitis 31 occasional (7.5%) HP:0012115
28 hematuria 31 occasional (7.5%) HP:0000790
29 purpura 31 occasional (7.5%) HP:0000979
30 gastrointestinal hemorrhage 31 occasional (7.5%) HP:0002239
31 osteoarthritis 31 occasional (7.5%) HP:0002758
32 goiter 31 occasional (7.5%) HP:0000853
33 papule 31 occasional (7.5%) HP:0200034
34 keratoconjunctivitis sicca 31 occasional (7.5%) HP:0001097
35 abnormality of bone marrow cell morphology 31 occasional (7.5%) HP:0005561
36 asplenia 31 occasional (7.5%) HP:0001746
37 abnormal blistering of the skin 31 occasional (7.5%) HP:0008066
38 pleural effusion 31 occasional (7.5%) HP:0002202
39 upper airway obstruction 31 occasional (7.5%) HP:0002781
40 polyneuropathy 31 occasional (7.5%) HP:0001271
41 aortic root aneurysm 31 occasional (7.5%) HP:0002616
42 xerostomia 31 occasional (7.5%) HP:0000217
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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.63 ACTC1 B2M CALR LYZ TTR VWF
2 digestive/alimentary MP:0005381 9.26 B2M LYZ TTR VWF
3 homeostasis/metabolism MP:0005376 9.1 ACTC1 B2M CALR LYZ TTR 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 221)
# 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
Caspofungin Approved Phase 3 162808-62-0, 179463-17-3 468682 2826718
5
Amphotericin B Approved, Investigational Phase 3 1397-89-3 14956 5280965
6
Dalteparin Approved Phase 3 9005-49-6
7
Tinzaparin Approved Phase 3 9041-08-1, 9005-49-6 25244225
8
Guaifenesin Approved, Investigational, Vet_approved Phase 3 93-14-1 3516
9
Morphine Approved, Investigational Phase 3 57-27-2 5288826
10
Darbepoetin alfa Approved, Investigational Phase 3 209810-58-2, 11096-26-7
11
Ondansetron Approved Phase 3 99614-02-5 4595
12
Iron Approved Phase 3 7439-89-6 23925 29936
13
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
14
Doxycycline Approved, Investigational, Vet_approved Phase 2, Phase 3 564-25-0 54671203
15
Lenalidomide Approved Phase 3 191732-72-6 216326
16
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
17
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
18
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
19
Daratumumab Approved Phase 3 945721-28-8
20
Bortezomib Approved, Investigational Phase 3 179324-69-7 387447 93860
21
Melphalan Approved Phase 3 148-82-3 4053 460612
22
Lenograstim Approved, Investigational Phase 3 135968-09-1
23
Sargramostim Approved, Investigational Phase 3 123774-72-1, 83869-56-1
24
Ginseng Approved, Investigational, Nutraceutical Phase 3 50647-08-0
25
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
26 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
27 Molgramostim Investigational Phase 3 99283-10-0
28 Anesthetics, Dissociative Phase 3
29 Excitatory Amino Acid Antagonists Phase 3
30 Hematinics Phase 3
31 Epoetin alfa Phase 3 113427-24-0
32 Antifungal Agents Phase 3
33 Amebicides Phase 3
34 Liposomal amphotericin B Phase 3
35 Heparin, Low-Molecular-Weight Phase 3
36 Fibrinolytic Agents Phase 3
37 Respiratory System Agents Phase 3
38 Antitussive Agents Phase 3
39 Chlorpheniramine, phenylpropanolamine drug combination Phase 3
40 Psychotropic Drugs Phase 3
41 Anti-Anxiety Agents Phase 3
42 Antipsychotic Agents Phase 3
43 Cola Phase 3
44 Liver Extracts Phase 3
45 ferric gluconate Phase 3
46 Iron Supplement Phase 3
47 Antiprotozoal Agents Phase 2, Phase 3
48 Antiparasitic Agents Phase 2, Phase 3
49 Antimalarials Phase 2, Phase 3
50 Pharmaceutical Solutions Phase 3

Interventional clinical trials:

(show top 50) (show all 176)
# 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 A Pilot Study to Evaluate 18F Florbetapir Binding to Cardiac Amyloid in Patients Undergoing Chemotherapy Recruiting NCT03333551 Phase 4 F18 Florbetapir (amyvid) cardiac PET/CT imaging
3 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
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 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
6 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
7 Stem Cell Transplant as Standard Therapy for Symptomatic Multiple Myeloma Completed NCT00004165 Phase 3 melphalan
8 Preparatory Aid to Improve Decision Making About Cancer Clinical Trials (PRE-ACT) Completed NCT00750009 Phase 3
9 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
10 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
11 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
12 Does Hypericum Reduce Fatigue in Cancer Patients on Chemotherapy? A Randomized, Double-Blind, Placebo-Controlled Clinical Trial Completed NCT00005805 Phase 3
13 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
14 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
15 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
16 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
17 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
18 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
19 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
20 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
21 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
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, 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)
24 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)
25 Frontline Lenalidomide for AL Amyloidosis Involving Myocardium: Investigation of Organ Reversing Capacity of Lenalidomide Recruiting NCT04298372 Phase 3 Lenalidomide 25mg
26 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
27 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
28 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
29 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
30 A Multicenter Phase II Trial of Bortezomib (Velcade), Melphalan, and Dexamethasone (V-MD) in Patients With Symptomatic AL-Amyloidosis or Light Chain Deposition Disease Unknown status NCT00520767 Phase 2 bortezomib;dexamethasone;melphalan
31 Myeloblative Therapy With Autologous Hematopoietic Stem Cell Transplantation in Patients With Multiple Myeloma and B-Cell Malignancies Unknown status NCT00003163 Phase 2 cyclophosphamide;melphalan
32 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
33 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
34 Phase II Trial of 4'-IODO-4'-Deoxydoxorubicin in Primary Amyloidosis (AL) Completed NCT00003853 Phase 2 4'-iodo-4'-deoxydoxorubicin
35 Phase II Study of Dexamethasone/Alpha-Interferon in AL Amyloidosis Completed NCT00002849 Phase 2 dexamethasone
36 A Phase II Trial of MRD (Melphalan, Lenalidomide and Dexamethasone) for Patients With AL Amyloidosis Completed NCT00679367 Phase 2 dexamethasone;lenalidomide;melphalan
37 A Phase II Trial of the Immunomodulatory Drug CC-5013 for Patients With AL Amyloidosis Completed NCT00091260 Phase 2 dexamethasone;lenalidomide
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 Autologous Peripheral Blood Stem Cell Transplantation With High Dose Melphalan For Treatment Of Primary Amyloidosis (AL) Completed NCT00002810 Phase 2 melphalan
40 Risk Adapted Intravenous Melphalan and Adjuvant Thalidomide and Dexamethasone for Untreated Patients With Primary Systemic Amyloidosis Completed NCT00089167 Phase 2 dexamethasone;melphalan;thalidomide
41 Phase 2 Study Of High Dose Chemotherapy Followed By Autologous Hematopoietic Stem Cell Support In Patients With Multiple Myeloma And Primary Light Chain Amyloidosis Completed NCT00007995 Phase 2 busulfan;cyclophosphamide;melphalan
42 A Phase II Trial of Lenalidomide (Revlimid®), Cyclophosphamide and Dexamethasone in Patients With Primary Systemic Amyloidosis Completed NCT00564889 Phase 2 cyclophosphamide;dexamethasone;lenalidomide
43 A Phase II Study of High-Dose Melphalan With Hematopoietic Stem Cell Reconstitution for Patients With Primary Systemic Amyloidosis Completed NCT00003353 Phase 2 melphalan
44 Therapeutic Potential of Human Immune Globulin Intravenous (IGIV) in Patients With Cardiac-Associated Light Chain (AL) Amyloidosis Completed NCT00547365 Phase 1, Phase 2
45 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
46 A Phase II Trial of Tandem Transplantation in AL Amyloidosis Completed NCT00075621 Phase 2 filgrastim;melphalan
47 Assessment of Early and Standard Intervention With Procrit® (Epoetin Alfa) 120,000 Units Once Every Three Weeks (Q3W) in Patients With Cancer Receiving Chemotherapy Completed NCT00255749 Phase 2
48 A Phase I/II Non-Myeloablative Allogeneic Hematopoietic Stem Cell Transplant for the Treatment of Patients With Hematologic Malignancies Using Busulfan, Fludarabine and Total Body Irradiation Completed NCT00245037 Phase 1, Phase 2 busulfan;cyclosporine;fludarabine phosphate;mycophenolate mofetil;Granulocyte colony-stimulating factor (G-CSF);Phenytoin;Methotrexate
49 OUTPATIENT SUBCUTANEOUS IL-2 AND ALPHA INTERFERON IN THE MANAGEMENT OF METASTATIC CANCER Completed NCT00002504 Phase 2
50 RC05CB A Pilot, Randomized Comparison of Standard Weekly Epoetin Alfa to Every-3-Week-Epoetin Alfa and Every 3-Week Darbepoetin Alfa Completed NCT00416624 Phase 2 darbepoetin alfa;epoetin alfa

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
Bone Marrow, Bone, Tongue, Spleen, Liver, Kidney, Heart
Sources

Publications for Al Amyloidosis

About this section

Articles related to Al Amyloidosis:

(show top 50) (show all 2578)
# Title Authors PMID Year
1
Coexistence of amyloidosis and light chain deposition disease in the heart. 61
33264681 2021
2
Characterization and prognostic implication of delayed complete response in AL amyloidosis. 61
33230879 2021
3
The impact of COVID-19 in the management of AL amyloidosis and Immunoglobulin Deposition Disease: A single-center experience. 61
33197297 2021
4
Prognostic restaging after treatment initiation in patients with AL amyloidosis. 61
33595624 2021
5
Cryo-EM reveals structural breaks in a patient-derived amyloid fibril from systemic AL amyloidosis. 61
33558536 2021
6
Changes in patient-reported outcomes in light chain amyloidosis in the first year after diagnosis and relationship to NT-proBNP change. 61
33563897 2021
7
Validation of the composite organ and hematologic response model for prognostic prediction in a Chinese light chain amyloidosis cohort. 61
33602009 2021
8
Coagulation Abnormalities in Light Chain Amyloidosis. 61
33549257 2021
9
Neuropathy associated with IgA monoclonal Gammopathy. A harbinger of AL amyloidosis. 61
33578240 2021
10
Giant Hepatomegaly with Spleno-testicular Enlargement in a Patient with Apolipoprotein A-I Amyloidosis: An Uncommon Type of Amyloidosis in Japan. 61
32999221 2021
11
Cardiac amyloidosis masquerading as acute coronary syndrome. 61
33602760 2021
12
Use of Biomarkers to Diagnose and Manage Cardiac Amyloidosis. 61
33527656 2021
13
Early events in light chain aggregation at physiological pH reveal new insights on assembly, stability, and aggregate dissociation. 61
33533277 2021
14
A safety review of drug treatments for patients with systemic immunoglobulin light chain (AL) amyloidosis. 61
33583294 2021
15
Minimal residual disease negativity by next-generation flow cytometry is associated with improved organ response in AL amyloidosis. 61
33594045 2021
16
Transcriptional heterogeneity of clonal plasma cells and immune evasion in immunoglobulin light chain amyloidosis. 61
33040275 2021
17
Daratumumab for relapsed AL amyloidosis-When cumulative real-world data precedes clinical trials: A multisite study and systematic literature review. 61
33090552 2021
18
A unique case of light chain (AL) amyloidosis masquerading as hypophosphatemic osteomalacia. 61
32910218 2021
19
Outcome of patients with severe AL amyloidosis and biopsy-proven renal involvement ineligible for bone marrow transplantation. 61
32472525 2021
20
Retroperitoneal involvement with light chain amyloidosis- case series and literature review. 61
33118418 2021
21
Amyloid cast nephropathy with systemic AL amyloidosis. 61
33222047 2021
22
A case of cardiac amyloidosis incidentally detected by bone scintigraphy. 61
33392354 2021
23
Two distinct mechanisms of neuropathy in immunoglobulin light chain (AL) amyloidosis. 61
33540321 2021
24
Ocular manifestation of giant cell arteritis vs AL-amyloidosis: similar presentations but different approaches. 61
32787554 2021
25
Outcomes of Heart Transplantation in Cardiac Amyloidosis Patients: A Single Center Experience. 61
32917391 2021
26
Serum Hevylite® assay in the differential diagnosis of patients with high suspicion of AL Amyloidosis. 61
33455086 2021
27
Neuromuscular amyloidosis: Unmasking the master of disguise. 61
33458861 2021
28
Autologous stem cell transplantation vs bortezomib based chemotheraphy for the first-line treatment of systemic light chain amyloidosis in the UK. 61
33460466 2021
29
[Primary generalized AL amyloidosis]. 61
33512125 2021
30
Clarification on the definition of complete haematologic response in light-chain (AL) amyloidosis. 61
33410355 2021
31
Liver injury during rivaroxaban treatment in a patient with AL amyloidosis. 61
33427957 2021
32
Predictors of cardiac involvement and survival in patients with primary systemic light-chain amyloidosis: roles of the clinical, chemical, and 3-D speckle tracking echocardiography parameters. 61
33478398 2021
33
Comparison of the current renal staging, progression and response criteria to predict renal survival in AL amyloidosis using a Mayo cohort. 61
33428787 2021
34
Molecular mechanism of amyloidogenic mutations in hypervariable regions of antibody light chains. 61
33508322 2021
35
The not so innocent bystander: an unusual cause of progressive breathlessness. 61
33479041 2021
36
In from the cold: M-protein light chain glycosylation is positively associated with cold agglutinin titer levels. 61
33502021 2021
37
Effect of O-glycosylation on amyloid fibril formation of the variable domain in the Vλ6 light chain mutant Wil. 61
33127550 2021
38
Renal transplant outcomes in amyloidosis. 61
33439995 2021
39
Novel imaging techniques using 18 F-florbetapir PET/MRI can guide fascicular nerve biopsy in amyloid multiple mononeuropathy. 61
33094511 2021
40
Venetoclax induces deep hematologic remissions in t(11;14) relapsed/refractory AL amyloidosis. 61
33431806 2021
41
A case of AL amyloidosis presenting with refractory ventricular fibrillation. 61
33552893 2021
42
A pharmacist's review of the treatment of systemic light chain amyloidosis. 61
33028132 2021
43
Effects of Amyloid Light-Chain Amyloidosis on Clinical Characteristics and Prognosis in Multiple Myeloma: A Single-Center Retrospective Study. 61
33603480 2021
44
Measurable residual disease in multiple myeloma and light chain amyloidosis: more than meets the eye. 61
33508994 2021
45
Menorrhagia due to uterine amyloidosis in familial Mediterranean fever: case-based review. 61
33052444 2021
46
[18F]-Florbetaben PET/CT for Differential Diagnosis Among Cardiac Immunoglobulin Light Chain, Transthyretin Amyloidosis, and Mimicking Conditions. 61
32771577 2021
47
AL amyloidosis presenting as inflammatory polyarthritis: a case report. 61
33269662 2021
48
Amyloid Deposition of the Bilateral Ureters in a Patient With Chronic Systemic AL Amyloidosis. 61
32866513 2020
49
Acquired FXIII deficiency and AL amyloidosis: A case of a rare association. 61
32839100 2020
50
The Impact of AL Amyloidosis: The Patient Experience. 61
33099433 2020
Sources

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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Pathways related to Al Amyloidosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Innate Immune System 65
Show member pathways
 12.95 VWF TTR LYZ CALR B2M
2
Antigen Presentation- Folding, assembly and peptide loading of class I MHC 65
10.02 CALR B2M
Sources

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 9.65 VWF TTR LYZ CALR B2M
2 focal adhesion GO:0005925 9.54 CALR B2M ACTC1
3 azurophil granule lumen GO:0035578 9.46 TTR LYZ
4 phagocytic vesicle membrane GO:0030670 9.43 CALR B2M
5 extracellular exosome GO:0070062 9.43 VWF TTR LYZ CALR B2M ACTC1
6 specific granule lumen GO:0035580 9.4 LYZ B2M
7 tertiary granule lumen GO:1904724 9.32 LYZ B2M
8 MHC class I peptide loading complex GO:0042824 9.16 CALR B2M
9 extracellular space GO:0005615 9.1 VWF TTR LYZ CALR B2M ACTC1

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

# Name GO ID Score Top Affiliating Genes
1 neutrophil degranulation GO:0043312 9.5 TTR LYZ B2M
2 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependent GO:0002479 9.37 CALR B2M
3 response to drug GO:0042493 9.33 CALR B2M ACTC1
4 retina homeostasis GO:0001895 9.26 LYZ B2M
5 antigen processing and presentation of peptide antigen via MHC class I GO:0002474 8.96 CALR B2M
6 cellular protein metabolic process GO:0044267 8.8 TTR LYZ B2M

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

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.26 VWF TTR LYZ B2M
2 chaperone binding GO:0051087 9.16 VWF CALR
3 hormone binding GO:0042562 8.62 TTR CALR
Sources

Sources for Al Amyloidosis

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
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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