MCID: ALM001
MIFTS: 55

Al Amyloidosis

Categories: Blood diseases, Bone diseases, Genetic diseases, Immune diseases, Metabolic diseases, Nephrological diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Al Amyloidosis

MalaCards integrated aliases for Al Amyloidosis:

Name: Al Amyloidosis 54
Primary Systemic Amyloidosis 54 74
Primary Amyloidosis 54 74
Hereditary Amyloid Nephropathy Due to Lysozyme Variant 60
Familial Amyloid Nephropathy Due to Lysozyme Variant 60
Hereditary Renal Amyloidosis Due to Lysozyme Variant 60
Familial Renal Amyloidosis Due to Lysozyme Variant 60
Immunoglobulin Deposition Disease 74
Primary Systemic Al Amyloidosis 54
Amyloidosis Primary Systemic 54
Light Chain Amyloidosis 54
Primary Al Amyloidosis 54
Systemic Al Amyloidsis 54
Lysozyme Amyloidosis 60
Amyloidosis Primary 56
Alys Amyloidosis 60
Amyloidosis Al 54

Characteristics:

Orphanet epidemiological data:

60
alys amyloidosis
Inheritance: Autosomal dominant; Prevalence: <1/1000000 (Worldwide);

Classifications:



Summaries for Al Amyloidosis

NIH Rare Diseases : 54 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 and amyloidosis aa. An important gene associated with Al Amyloidosis is LYZ (Lysozyme), and among its related pathways/superpathways is Innate Immune System. The drugs Ketamine and Dexamethasone have been mentioned in the context of this disorder. Affiliated tissues include bone, kidney and bone marrow, and related phenotypes are fatigue and hypertrophic cardiomyopathy

Wikipedia : 77 Amyloid light-chain (AL) amyloidosis, also known as primary amyloidosis, is the most common form of... more...

Related Diseases for Al Amyloidosis

Diseases in the Amyloidosis family:

Amyloidosis, Hereditary, Transthyretin-Related 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 244)
# Related Disease Score Top Affiliating Genes
1 amyloidosis 31.2 B2M LYZ TTR
2 amyloidosis aa 30.0 B2M TTR
3 carpal tunnel syndrome 29.8 B2M TTR
4 amyloid tumor 29.7 B2M TTR
5 purpura 29.7 ADAMTS13 VWF
6 thrombosis 29.1 ADAMTS13 VWF
7 hereditary amyloidosis 29.1 B2M LYZ TTR
8 amyloidosis, familial visceral 28.9 B2M LYZ TTR
9 primary cutaneous amyloidosis 11.9
10 myeloma, multiple 11.8
11 primary localized amyloidosis 11.6
12 lichen amyloidosis 11.4
13 light chain deposition disease 11.3
14 amyloidosis, primary localized cutaneous, 1 11.1
15 amyloidosis, primary localized cutaneous, 2 11.1
16 amyloidosis, primary localized cutaneous, 3 11.1
17 amyloidosis, hereditary, transthyretin-related 11.0
18 factor x deficiency 10.2
19 spondylocarpotarsal synostosis syndrome 10.2
20 nephrotic syndrome 10.2
21 lymphoma 10.1
22 macroglobulinemia 10.1
23 tibial neuropathy 10.1 B2M TTR
24 tarsal tunnel syndrome 10.1 B2M TTR
25 median rhomboid glossitis 10.1 B2M TTR
26 nerve compression syndrome 10.1 B2M TTR
27 fanconi-like syndrome 10.1 B2M LGALS3
28 bombay phenotype 10.1 ADAMTS13 VWF
29 cholestasis 10.1
30 neuropathy 10.1
31 uremia 10.1 B2M VWF
32 primary thrombocytopenia 10.1 ADAMTS13 VWF
33 autoimmune disease of blood 10.0 ADAMTS13 VWF
34 von willebrand disease, type 2 10.0 ADAMTS13 VWF
35 blood coagulation disease 10.0 ADAMTS13 VWF
36 thrombotic thrombocytopenic purpura 10.0 ADAMTS13 VWF
37 leukemia 10.0
38 muscle disorders 10.0
39 myopathy 10.0
40 monoclonal gammopathy of uncertain significance 10.0
41 rere-related disorders 10.0
42 depression 10.0
43 von willebrand's disease 10.0 ADAMTS13 VWF
44 blood platelet disease 10.0 ADAMTS13 VWF
45 acute kidney failure 10.0 ADAMTS13 B2M
46 extramedullary plasmacytoma 10.0
47 hemolytic uremic syndrome, atypical 1 10.0 ADAMTS13 VWF
48 antiphospholipid syndrome 10.0 ADAMTS13 VWF
49 macroglossia 10.0
50 rheumatoid arthritis 10.0

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



Diseases related to Al Amyloidosis

Symptoms & Phenotypes for Al Amyloidosis

Human phenotypes related to Al Amyloidosis:

33 (show top 50) (show all 53)
# Description HPO Frequency HPO Source Accession
1 fatigue 33 hallmark (90%) HP:0012378
2 hypertrophic cardiomyopathy 33 hallmark (90%) HP:0001639
3 hepatomegaly 33 frequent (33%) HP:0002240
4 renal insufficiency 33 frequent (33%) HP:0000083
5 proteinuria 33 frequent (33%) HP:0000093
6 nephropathy 33 frequent (33%) HP:0000112
7 arthralgia 33 frequent (33%) HP:0002829
8 dyspnea 33 frequent (33%) HP:0002094
9 congestive heart failure 33 frequent (33%) HP:0001635
10 nephrotic syndrome 33 frequent (33%) HP:0000100
11 lymphadenopathy 33 frequent (33%) HP:0002716
12 enlarged kidney 33 frequent (33%) HP:0000105
13 abnormality of chromosome segregation 33 frequent (33%) HP:0002916
14 interstitial pulmonary abnormality 33 frequent (33%) HP:0006530
15 abnormal ekg 33 frequent (33%) HP:0003115
16 elevated alkaline phosphatase 33 frequent (33%) HP:0003155
17 pulmonary edema 33 frequent (33%) HP:0100598
18 chronic constipation 33 frequent (33%) HP:0012450
19 acute infectious pneumonia 33 frequent (33%) HP:0011949
20 monoclonal immunoglobulin m proteinemia 33 frequent (33%) HP:0005508
21 multiple myeloma 33 frequent (33%) HP:0006775
22 arthropathy 33 frequent (33%) HP:0003040
23 pedal edema 33 frequent (33%) HP:0010741
24 osteoarthritis 33 occasional (7.5%) HP:0002758
25 hypertension 33 occasional (7.5%) HP:0000822
26 macroglossia 33 occasional (7.5%) HP:0000158
27 malabsorption 33 occasional (7.5%) HP:0002024
28 hepatitis 33 occasional (7.5%) HP:0012115
29 abnormal blistering of the skin 33 occasional (7.5%) HP:0008066
30 decreased antibody level in blood 33 occasional (7.5%) HP:0004313
31 goiter 33 occasional (7.5%) HP:0000853
32 keratoconjunctivitis sicca 33 occasional (7.5%) HP:0001097
33 hematuria 33 occasional (7.5%) HP:0000790
34 xerostomia 33 occasional (7.5%) HP:0000217
35 purpura 33 occasional (7.5%) HP:0000979
36 gastrointestinal hemorrhage 33 occasional (7.5%) HP:0002239
37 papule 33 occasional (7.5%) HP:0200034
38 asplenia 33 occasional (7.5%) HP:0001746
39 abnormality of bone marrow cell morphology 33 occasional (7.5%) HP:0005561
40 polyneuropathy 33 occasional (7.5%) HP:0001271
41 upper airway obstruction 33 occasional (7.5%) HP:0002781
42 pleural effusion 33 occasional (7.5%) HP:0002202
43 orthostatic hypotension due to autonomic dysfunction 33 occasional (7.5%) HP:0004926
44 adrenal insufficiency 33 occasional (7.5%) HP:0000846
45 skin nodule 33 occasional (7.5%) HP:0200036
46 autonomic bladder dysfunction 33 occasional (7.5%) HP:0005341
47 intermittent claudication 33 occasional (7.5%) HP:0004417
48 bradycardia 33 occasional (7.5%) HP:0001662
49 abnormality of the submandibular glands 33 occasional (7.5%) HP:0010287
50 mechanical ileus 33 occasional (7.5%) HP:0010676

MGI Mouse Phenotypes related to Al Amyloidosis:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.77 B2M LGALS3 LYZ TTR VWF
2 digestive/alimentary MP:0005381 9.72 B2M LGALS3 LYZ TTR VWF
3 hematopoietic system MP:0005397 9.65 ADAMTS13 B2M LGALS3 LYZ VWF
4 homeostasis/metabolism MP:0005376 9.63 ADAMTS13 B2M LGALS3 LYZ TTR VWF
5 immune system MP:0005387 9.35 ADAMTS13 B2M LGALS3 LYZ VWF
6 no phenotypic analysis MP:0003012 8.92 ADAMTS13 LYZ TTR VWF

Drugs & Therapeutics for Al Amyloidosis

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

(show top 50) (show all 268)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ketamine Approved, Vet_approved Phase 3 6740-88-1 3821
2
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 1,Phase 2,Not Applicable 50-02-2 5743
3
Melphalan Approved Phase 3,Phase 2,Phase 1,Not Applicable 148-82-3 460612 4053
4
Lenograstim Approved, Investigational Phase 3,Phase 2,Phase 1 135968-09-1
5
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3,Phase 1,Phase 2,Not Applicable 1177-87-3
6
Cyclophosphamide Approved, Investigational Phase 3,Phase 1,Phase 2,Not Applicable 50-18-0, 6055-19-2 2907
7
Lenalidomide Approved Phase 3,Phase 1,Phase 2,Not Applicable 191732-72-6 216326
8
Sargramostim Approved, Investigational Phase 3,Phase 2,Phase 1 123774-72-1, 83869-56-1
9
Bortezomib Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable 179324-69-7 387447 93860
10
Mechlorethamine Approved, Investigational Phase 3,Phase 1,Phase 2,Not Applicable 51-75-2 4033
11
Iron Approved, Experimental Phase 3,Phase 1,Phase 2 7439-89-6, 15438-31-0 27284 23925
12
Darbepoetin alfa Approved, Investigational Phase 3,Phase 2 209810-58-2, 11096-26-7
13
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
14
Dalteparin Approved Phase 3 9005-49-6
15
Heparin Approved, Investigational Phase 3 9005-49-6 46507594 772
16
Ondansetron Approved Phase 3 99614-02-5 4595
17
Caspofungin Approved Phase 3,Phase 2 162808-62-0, 179463-17-3 2826718 468682
18
Amphotericin B Approved, Investigational Phase 3 1397-89-3 5280965 14956
19
Miconazole Approved, Investigational, Vet_approved Phase 3,Phase 1,Phase 2,Not Applicable 22916-47-8 4189
20
Guaifenesin Approved, Investigational, Vet_approved Phase 3,Not Applicable 93-14-1 3516
21
Dextromethorphan Approved Phase 3 125-71-3 5362449 5360696
22
Morphine Approved, Investigational Phase 3,Not Applicable 57-27-2 5288826
23
Doxycycline Approved, Investigational, Vet_approved Phase 2, Phase 3,Not Applicable 564-25-0 54671203
24
Ixazomib Approved, Investigational Phase 3,Phase 1,Phase 2 1072833-77-2
25 Daratumumab Approved Phase 3,Phase 1,Phase 2 945721-28-8
26
Ginseng Approved, Investigational, Nutraceutical Phase 3 50647-08-0
27
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
28
Glycine Approved, Nutraceutical, Vet_approved Phase 3,Phase 1,Phase 2 56-40-6 750
29 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
30 Peripheral Nervous System Agents Phase 3,Phase 1,Phase 2,Not Applicable
31 Excitatory Amino Acids Phase 3
32 Anesthetics Phase 3
33 Anesthetics, Dissociative Phase 3
34 Neurotransmitter Agents Phase 3,Phase 1,Phase 2
35 Excitatory Amino Acid Antagonists Phase 3
36 Analgesics Phase 3,Phase 2,Not Applicable
37 Anesthetics, Intravenous Phase 3
38 Anesthetics, General Phase 3
39 Central Nervous System Depressants Phase 3,Phase 1,Phase 2,Not Applicable
40 Antineoplastic Agents, Alkylating Phase 3,Phase 1,Phase 2,Not Applicable
41 BB 1101 Phase 3,Phase 1,Phase 2,Not Applicable
42 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 1,Phase 2,Not Applicable
43 Antiemetics Phase 3,Phase 1,Phase 2,Not Applicable
44 Immunologic Factors Phase 3,Phase 1,Phase 2,Not Applicable
45 Gastrointestinal Agents Phase 3,Phase 1,Phase 2,Not Applicable
46 glucocorticoids Phase 3,Phase 1,Phase 2,Not Applicable
47 Immunosuppressive Agents Phase 3,Phase 1,Phase 2,Not Applicable
48 HIV Protease Inhibitors Phase 3,Phase 1,Phase 2,Not Applicable
49 Alkylating Agents Phase 3,Phase 1,Phase 2,Not Applicable
50 Antineoplastic Agents, Hormonal Phase 3,Phase 1,Phase 2,Not Applicable

Interventional clinical trials:

(show top 50) (show all 187)
# Name Status NCT ID Phase Drugs
1 Cardiac Uptake of 18F Florbetapir in Patients Undergoing Chemotherapy Not yet recruiting NCT03333551 Phase 4 F18 Florbetapir (amyvid) cardiac PET/CT imaging
2 Ketamine Hydrochloride and Best Pain Management in Treating Cancer Patients With Neuropathic Pain Unknown status NCT01316744 Phase 3 ketamine hydrochloride
3 Bortezomib and Dexamethasone Followed by ASCT Compared With ASCT Alone in Treating Patients With AL Amyloidosis Completed NCT01998503 Phase 3 Bortezomib;dexamethasone;Melphalan
4 Intensive Versus Conventional Treatment in Patients With Primary Amyloidosis Completed NCT00344526 Phase 3 Melphalan;Dexamethasone
5 Low-Dose Melphalan and Dexamethasone Compared With High-Dose Melphalan Followed By Autologous Stem Cell Transplant in Treating Patients With Primary Systemic Amyloidosis Completed NCT00477971 Phase 3 dexamethasone;melphalan
6 Melphalan and Dexamethasone With or Without Bortezomib in Treating Patients With Previously Untreated Systemic Light-Chain Amyloidosis Completed NCT01078454 Phase 3 melphalan;dexamethasone;bortezomib
7 A Trial for Systemic Light-chain (AL) Amyloidosis Completed NCT01277016 Phase 3 BMDex
8 Melphalan Followed by Peripheral Stem Cell Transplantation in Treating Patients With Multiple Myeloma Completed NCT00004165 Phase 3 melphalan
9 Personalized Information or Basic Information in Helping Patients Make Decisions About Participating in a Clinical Trial Completed NCT00750009 Phase 3
10 American Ginseng in Treating Patients With Fatigue Caused by Cancer Completed NCT00719563 Phase 3 American ginseng
11 Opioid Titration Order Sheet or Standard Care in Treating Patients With Cancer Pain Completed NCT00666211 Phase 3
12 Darbepoetin Alfa With or Without Iron in Treating Anemia Caused By Chemotherapy in Patients With Cancer Completed NCT00661999 Phase 3 sodium ferric gluconate complex in sucrose
13 Fentanyl Sublingual Spray in Treating Patients With Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
14 St. John's Wort in Relieving Fatigue in Patients Undergoing Chemotherapy or Hormone Therapy for Cancer Completed NCT00005805 Phase 3
15 Dalteparin to Prevent Complications in Cancer Patients Receiving Chemotherapy Through a Catheter Completed NCT00006083 Phase 3 Fragmin
16 Ondansetron in Treating Patients With Advanced Cancer and Chronic Nausea and Vomiting Not Caused by Cancer Treatment Completed NCT00006348 Phase 3 ondansetron
17 Darbepoetin Alfa Compared With Epoetin Alfa in Treating Anemia in Patients Receiving Chemotherapy for Cancer Completed NCT00070382 Phase 3 darbepoetin alfa;epoetin alfa
18 Caspofungin Acetate Compared With Amphotericin B Liposomal in Treating Patients With Persistent Fever and Neutropenia Following Cancer Treatment Completed NCT00008359 Phase 3 caspofungin acetate;liposomal amphotericin B
19 Liposomal Amphotericin B in Treating Granulocytopenia and Persistent Unexplained Fever in Cancer Patients Completed NCT00003938 Phase 3 liposomal amphotericin B
20 Treatment for Chronic Pain in Patients With Advanced Cancer Completed NCT00003687 Phase 3 dextromethorphan hydrobromide;morphine sulfate
21 Study of the Safety and Efficacy of NC-503 in Secondary (AA) Amyloidosis Completed NCT00035334 Phase 2, Phase 3 NC-503 (Anti-amyloidotic (AA) Agent)
22 A 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 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 Amyloid Light-chain (AL) Amyloidosis Recruiting NCT03201965 Phase 3 Cyclophosphamide;Bortezomib;Dexamethasone, 40 mg;Daratumumab
24 Study of Dexamethasone Plus IXAZOMIB (MLN9708) or Physicians Choice of Treatment in Relapsed or Refractory Systemic Light Chain (AL) Amyloidosis Recruiting NCT01659658 Phase 3 IXAZOMIB;Dexamethasone;Melphalan;Cyclophosphamide;Thalidomide;Lenalidomide
25 Assessment of 18F-Florbetaben Whole-body PET for the Detection of Cardiac and Extracardiac Sites of Amyloid Deposits Not yet recruiting NCT03616496 Phase 2, Phase 3 Neuraceq 300MBq/mL Solution for Injection for PET imaging
26 Trial of High Dose Melphalan/Stem Cell Transplant With or Without Bortezomib Terminated NCT02489500 Phase 3 Bortezomib;Melphalan;Neupogen
27 The VITAL Amyloidosis Study, a Global Phase 3, Efficacy and Safety Study of NEOD001 in Patients With AL Amyloidosis Terminated NCT02312206 Phase 3 NEOD001
28 A Dose Escalation Study of Carfilzomib Taken With Thalidomide and Dexamethasone in Relapsed AL Amyloidosis Unknown status NCT02545907 Phase 1, Phase 2 Carfilzomib;Thalidomide;Dexamethasone
29 Lenalidomide, Dexamethasone and Cyclophosphamide in Amyloidosis (AL) Unknown status NCT00981708 Phase 1, Phase 2 Lenalidomide, Dexamethasone and Cyclophosphamide
30 Peripheral Stem Cell Transplantation in Treating Patients With Multiple Myeloma or Other B-cell Cancers Unknown status NCT00003163 Phase 2 cyclophosphamide;melphalan
31 Pomalidomide and Dexamethasone (PDex) in AL Amyloidosis Completed NCT01510613 Phase 2 Pomalidomide and Dexamethasone
32 Human Immune Globulin in Treating Patients With Primary Amyloidosis That is Causing Heart Dysfunction Completed NCT00547365 Phase 1, Phase 2
33 A Trial of Treatment With Lenalidomide-Melphalan-Dexamethasone in Patients With Primary (AL) Amyloidosis Completed NCT00883623 Phase 2 Lenalidomide
34 Melphalan, Lenalidomide, and Dexamethasone in Treating Patients With Primary Systemic Amyloidosis Completed NCT00679367 Phase 2 dexamethasone;lenalidomide;melphalan
35 CC-5013 With or Without Dexamethasone in Treating Patients With Primary Systemic Amyloidosis Completed NCT00091260 Phase 2 dexamethasone;lenalidomide
36 S9628 Dexamethasone Plus Interferon Alfa in Treating Patients With Primary Systemic Amyloidosis Completed NCT00002849 Phase 2 dexamethasone
37 Bortezomib, Cyclophosphamide, and Dexamethasone in Treating Patients With Primary Systemic Light Chain Amyloidosis Completed NCT01072773 Phase 2 bortezomib;cyclophosphamide;dexamethasone
38 Melphalan, Bortezomib, and Stem Cell Transplant in Treating Patients With Primary Systemic Amyloidosis Completed NCT00790647 Phase 2 bortezomib;melphalan
39 Efficacy of CC-5013 (Revlimid or Lenalidomide) in Patients With Primary Systemic Amyloidosis Completed NCT00166413 Phase 2 CC-5013
40 Cyclophosphamide, Lenalidomide and Dexamethasone (CLD) for Previously Treated Patients With AL Amyloidosis Completed NCT00607581 Phase 2 cyclophosphamide;lenalidomide;dexamethasone
41 4'-Iodo-4'-Deoxydoxorubicin in Treating Patients With Primary Systemic Amyloidosis Completed NCT00003853 Phase 2 4'-iodo-4'-deoxydoxorubicin
42 Lenalidomide, Cyclophosphamide, and Dexamethasone in Treating Patients With Primary Systemic Amyloidosis Completed NCT00564889 Phase 2 cyclophosphamide;dexamethasone;lenalidomide
43 Melphalan, Thalidomide, and Dexamethasone in Treating Patients With Newly Diagnosed, Previously Untreated Primary Systemic Amyloidosis Completed NCT00089167 Phase 2 dexamethasone;melphalan;thalidomide
44 Lendexal in Patients With Primary Systemic Amyloidosis (AL) Newly Diagnosed Completed NCT01194791 Phase 2 Lenalidomide;Cyclophosphamide;Dexamethasone
45 Phase 1/2, Open Label, Dose Escalation Study of NEOD001 in Subjects With Light Chain (AL) Amyloidosis Completed NCT01707264 Phase 1, Phase 2 NEOD001
46 S0115, High-Dose Melphalan and Autologous Peripheral Stem Cell Transplantation in Treating Patients With Multiple Myeloma or Primary Systemic Amyloidosis Completed NCT00064337 Phase 2 cyclophosphamide;dexamethasone;melphalan;thalidomide
47 High-Dose Melphalan Plus Peripheral Stem Cell Transplantation in Treating Patients With Primary Systemic Amyloidosis Completed NCT00003353 Phase 2 melphalan
48 The PRONTO Study, a Global Phase 2b Study of NEOD001 in Previously Treated Subjects With Light Chain (AL) Amyloidosis Completed NCT02632786 Phase 2 NEOD001;Placebo
49 Chemotherapy Plus Peripheral Stem Cell Transplant in Treating Patients Who Have Multiple Myeloma or Primary Systemic Amyloidosis Completed NCT00007995 Phase 2 busulfan;cyclophosphamide;melphalan
50 A Trial for the Treatment of Cardiac AL-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

Genetic Tests for Al Amyloidosis

Anatomical Context for Al Amyloidosis

MalaCards organs/tissues related to Al Amyloidosis:

42
Bone, Kidney, Bone Marrow, Liver, Heart, Skin, Testes

Publications for Al Amyloidosis

Articles related to Al Amyloidosis:

(show top 50) (show all 1011)
# Title Authors Year
1
Pulmonary AL- amyloidosis masquerading as lung malignancy in an Australian Indigenous patient with Sjogren's syndrome. ( 30560052 )
2019
2
AL amyloidosis cardiac staging updated using BNP. ( 30655301 )
2019
3
BNP-based staging system in AL amyloidosis. ( 30655307 )
2019
4
Performance analysis of AL amyloidosis cardiac biomarker staging systems with special focus on renal failure and atrial arrhythmia. ( 30655373 )
2019
5
Symptomatic involvement of the stomach and duodenum as initial presentation of AL amyloidosis. ( 30659008 )
2019
6
High-dose melphalan and autologous peripheral blood stem cell transplantation in patients with AL amyloidosis and cardiac defibrillators. ( 30664726 )
2019
7
Improved outcomes for kidney transplantation in AL amyloidosis: impact on practice. ( 30665564 )
2019
8
When should treatment of AL amyloidosis start at relapse? Early, to prevent organ progression. ( 30670537 )
2019
9
Delay treatment of AL amyloidosis at relapse until symptomatic: devil is in the details. ( 30670538 )
2019
10
Comparative analysis of staging systems in AL amyloidosis. ( 30675009 )
2019
11
AL amyloidosis with a localized B cell neoplasia. ( 30680453 )
2019
12
Incidence of AL Amyloidosis in Olmsted County, Minnesota, 1990 through 2015. ( 30713046 )
2019
13
Prognostic restaging at the time of second-line therapy in patients with AL amyloidosis. ( 30737485 )
2019
14
Hypocalcemia and hypophosphatemia after treatment with zoledronic acid in a patient with AL amyloidosis. ( 30771083 )
2019
15
Monoclonal Gammopathy Plus Positive Amyloid Biopsy Does Not Always Equal AL Amyloidosis. ( 30773682 )
2019
16
Associations between Health-Related Quality of Life and Self-Reported Emergency Room Department Visits and Inpatient Hospitalizations: Insights from a Secondary Data Analysis of Patients with Light-Chain (AL) Amyloidosis. ( 30796725 )
2019
17
Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease. ( 30879471 )
2019
18
Radiologically Guided Renal Artery Embolization with an Amplatzer Vascular Plug as a Rescue Therapy for Refractory Nephrotic Syndrome in AL-Amyloidosis. ( 30886753 )
2019
19
Cryo-EM structure of cardiac amyloid fibrils from an immunoglobulin light chain AL amyloidosis patient. ( 30894521 )
2019
20
Cryo-EM structure of a light chain-derived amyloid fibril from a patient with systemic AL amyloidosis. ( 30894526 )
2019
21
Non-proteinuric renal al amyloidosis in waldenström's macroglobulinemia. ( 30919534 )
2019
22
Regression of chronic kidney disease in a patient with AL amyloidosis: A case report
. ( 30935458 )
2019
23
Splenic rupture secondary to amyloid light-chain (AL) amyloidosis associated with multiple myeloma. ( 30949329 )
2019
24
Early Detection of Multiorgan Light Chain (AL) Amyloidosis by Whole Body 18F-florbetapir PET/CT. ( 30954943 )
2019
25
Autologous stem cell transplantation in patients with AL amyloidosis with impaired renal function. ( 30962503 )
2019
26
Comparison of different techniques to identify cardiac involvement in immunoglobulin light chain (AL) amyloidosis. ( 30975646 )
2019
27
Breast MALT lymphoma and AL amyloidosis complicating Sjögren's syndrome. ( 30975774 )
2019
28
Long term outcome of patients treated on clinical trials of immunomodulatory agents for the treatment of AL amyloidosis: A pooled analysis. ( 31020691 )
2019
29
Daratumumab proves safe and highly effective in AL amyloidosis. ( 29938774 )
2019
30
High-dose melphalan and stem cell transplantation in systemic AL amyloidosis in the era of novel anti-plasma cell therapy: a comprehensive review. ( 30089901 )
2019
31
Chronic intestinal pseudo-obstruction due to al amyloidosis: a case report and literature review. ( 30232705 )
2019
32
Optimizing deep response assessment for AL amyloidosis using involved free light chain level at end of therapy: failure of the serum free light chain ratio. ( 30258095 )
2019
33
Daratumumab-based therapy in patients with heavily-pretreated AL amyloidosis. ( 30267007 )
2019
34
Long-term response to daratumumab in a patient with advanced immunoglobulin light-chain (AL) amyloidosis with organ damage. ( 30488327 )
2019
35
Primary Systemic Amyloidosis Mimicking Rhinophyma. ( 30984606 )
2019
36
Primary Systemic Amyloidosis. ( 30775311 )
2019
37
Induction Therapy with Bortezomib and Dexamethasone and Conditioning with High-Dose Melphalan and Bortezomib Followed by Autologous Stem Cell Transplantation for AL Amyloidosis: Long Term Follow-Up Analysis. ( 30639823 )
2019
38
Primary systemic amyloidosis in patients with Waldenström macroglobulinemia. ( 30315235 )
2019
39
Usefulness of gastroduodenal biopsy in the differential diagnosis of systemic AH amyloidosis from systemic AL amyloidosis. ( 29660165 )
2018
40
Kidney embolization induces prompt organ response in a 86-year-old patient with MGRS-related AL-amyloidosis. ( 30548910 )
2018
41
Grading cardiac response in AL amyloidosis: implications for relapse and survival. ( 30569572 )
2018
42
Long-term outcome of kidney transplantation in AL amyloidosis. ( 30580886 )
2018
43
A pilot study demonstrating cardiac uptake with 18F-florbetapir PET in AL amyloidosis patients with cardiac involvement. ( 30661419 )
2018
44
Efficacy of lenalidomide as salvage therapy for patients with AL amyloidosis. ( 30663408 )
2018
45
Clinical observation of a combination of diffuse alveolar-septal pulmonary lesion and amyloid cardiomyopathy in systemic AL-amyloidosis in the elderly. ( 30701840 )
2018
46
AL amyloidosis, hypothyroidism and reduced tissue availability of thyroid hormones by thyroid hormone-binding immunoglobulin: a new possible perspective. ( 28805259 )
2018
47
Light Chain (AL) Amyloidosis: The Journey to Diagnosis. ( 28808991 )
2018
48
Pathology and diagnosis of renal non-AL amyloidosis. ( 28828707 )
2018
49
Real world data of the impact of first cycle daratumumab on multiple myeloma and AL amyloidosis services. ( 28832959 )
2018
50
Unravelling hypothyroidism in AL amyloidosis: Authors' reply. ( 28861923 )
2018

Variations for Al Amyloidosis

Expression for Al Amyloidosis

Search GEO for disease gene expression data for Al Amyloidosis.

Pathways for Al Amyloidosis

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.62 B2M LGALS3 LYZ TTR VWF

GO Terms for Al Amyloidosis

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

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 9.58 ADAMTS13 B2M LGALS3
2 extracellular exosome GO:0070062 9.55 B2M LGALS3 LYZ TTR VWF
3 azurophil granule lumen GO:0035578 9.37 LYZ TTR
4 extracellular space GO:0005615 9.35 ADAMTS13 B2M LGALS3 LYZ TTR
5 specific granule lumen GO:0035580 9.32 B2M LYZ
6 tertiary granule lumen GO:1904724 9.26 B2M LYZ
7 extracellular region GO:0005576 9.1 ADAMTS13 B2M LGALS3 LYZ TTR VWF

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

# Name GO ID Score Top Affiliating Genes
1 platelet activation GO:0030168 9.32 ADAMTS13 VWF
2 hemostasis GO:0007599 9.26 ADAMTS13 VWF
3 retina homeostasis GO:0001895 9.16 B2M LYZ
4 cellular protein metabolic process GO:0044267 9.13 B2M LYZ TTR
5 neutrophil degranulation GO:0043312 8.92 B2M LGALS3 LYZ TTR

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

# Name GO ID Score Top Affiliating Genes
1 integrin binding GO:0005178 8.96 ADAMTS13 VWF
2 identical protein binding GO:0042802 8.92 B2M LYZ TTR VWF

Sources for Al Amyloidosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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