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MCID: ALM001
MIFTS: 60

Al Amyloidosis

Categories: Blood diseases, Bone diseases, Genetic diseases, Immune diseases, Metabolic 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 53
Primary Systemic Amyloidosis 53 73
Primary Amyloidosis 53 73
Hereditary Amyloid Nephropathy Due to Lysozyme Variant 59
Familial Amyloid Nephropathy Due to Lysozyme Variant 59
Hereditary Renal Amyloidosis Due to Lysozyme Variant 59
Familial Renal Amyloidosis Due to Lysozyme Variant 59
Immunoglobulin Deposition Disease 73
Primary Systemic Al Amyloidosis 53
Amyloidosis Primary Systemic 53
Light Chain Amyloidosis 53
Primary Al Amyloidosis 53
Systemic Al Amyloidsis 53
Lysozyme Amyloidosis 59
Amyloidosis Primary 55
Alys Amyloidosis 59
Amyloidosis Al 53

Characteristics:

Orphanet epidemiological data:

59
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
See all MalaCards categories (disease lists)
ICD10: 34 33
Orphanet: 59  
Rare renal diseases
Rare systemic and rhumatological diseases


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Summaries for Al Amyloidosis

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NIH Rare Diseases : 53 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 acetate have been mentioned in the context of this disorder. Affiliated tissues include bone, liver and heart, and related phenotypes are osteoarthritis and hypertension

Wikipedia : 76 Amyloid light-chain (AL) amyloidosis, primary systemic amyloidosis (PSA) or just primary amyloidosis is... more...

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Related Diseases for Al Amyloidosis

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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 203)
# Related Disease Score Top Affiliating Genes
1 amyloidosis 31.3 B2M LYZ TTR
2 amyloidosis aa 29.8 B2M TTR
3 amyloid tumor 29.5 B2M TTR
4 hereditary amyloidosis 29.1 B2M LYZ TTR
5 primary cutaneous amyloidosis 11.9
6 myeloma, multiple 11.7
7 lichen amyloidosis 11.4
8 light chain deposition disease 11.3
9 primary localized amyloidosis 11.2
10 amyloidosis, primary localized cutaneous, 1 11.0
11 amyloidosis, primary localized cutaneous, 2 11.0
12 amyloidosis, primary localized cutaneous, 3 11.0
13 amyloidosis, hereditary, transthyretin-related 10.9
14 factor x deficiency 10.2
15 nephrotic syndrome 10.1
16 lymphoma 10.1
17 macroglobulinemia 10.1
18 cholestasis 10.1
19 neuropathy 10.1
20 tibial neuropathy 10.0 B2M TTR
21 myopathy 10.0
22 monoclonal gammopathy of uncertain significance 10.0
23 depression 10.0
24 tarsal tunnel syndrome 10.0 B2M TTR
25 median rhomboid glossitis 10.0 B2M TTR
26 nerve compression syndrome 10.0 B2M TTR
27 sjogren syndrome 10.0
28 spondylocarpotarsal synostosis syndrome 10.0
29 nodular cutaneous amyloidosis 10.0
30 macroglossia 9.9
31 rheumatoid arthritis 9.9
32 temporal arteritis 9.9
33 infantile liver failure syndrome 1 9.9
34 arthritis 9.9
35 glomerulonephritis 9.9
36 leukemia 9.9
37 uremia 9.9 B2M VWF
38 carpal tunnel syndrome 9.9 B2M TTR
39 diffuse glomerulonephritis 9.9 B2M LYZ
40 constrictive pericarditis 9.9
41 pericarditis 9.9
42 cutis laxa 9.9
43 acquired cutis laxa 9.9
44 pulmonary sarcoidosis 9.9
45 inflammatory bowel disease 9.9
46 gastritis 9.9
47 lymphangiosarcoma 9.9 LYZ VWF
48 autoimmune disease 9.9
49 anxiety 9.9
50 b-cell lymphomas 9.9

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:

32 (show top 50) (show all 53)
# Description HPO Frequency HPO Source Accession
1 osteoarthritis 32 occasional (7.5%) HP:0002758
2 hypertension 32 occasional (7.5%) HP:0000822
3 macroglossia 32 occasional (7.5%) HP:0000158
4 hepatomegaly 32 frequent (33%) HP:0002240
5 malabsorption 32 occasional (7.5%) HP:0002024
6 renal insufficiency 32 frequent (33%) HP:0000083
7 proteinuria 32 frequent (33%) HP:0000093
8 nephropathy 32 frequent (33%) HP:0000112
9 fatigue 32 hallmark (90%) HP:0012378
10 arthralgia 32 frequent (33%) HP:0002829
11 hypertrophic cardiomyopathy 32 hallmark (90%) HP:0001639
12 dyspnea 32 frequent (33%) HP:0002094
13 hepatitis 32 occasional (7.5%) HP:0012115
14 abnormal blistering of the skin 32 occasional (7.5%) HP:0008066
15 congestive heart failure 32 frequent (33%) HP:0001635
16 decreased antibody level in blood 32 occasional (7.5%) HP:0004313
17 nephrotic syndrome 32 frequent (33%) HP:0000100
18 goiter 32 occasional (7.5%) HP:0000853
19 keratoconjunctivitis sicca 32 occasional (7.5%) HP:0001097
20 hematuria 32 occasional (7.5%) HP:0000790
21 xerostomia 32 occasional (7.5%) HP:0000217
22 purpura 32 occasional (7.5%) HP:0000979
23 gastrointestinal hemorrhage 32 occasional (7.5%) HP:0002239
24 papule 32 occasional (7.5%) HP:0200034
25 lymphadenopathy 32 frequent (33%) HP:0002716
26 asplenia 32 occasional (7.5%) HP:0001746
27 abnormality of bone marrow cell morphology 32 occasional (7.5%) HP:0005561
28 enlarged kidney 32 frequent (33%) HP:0000105
29 edema of the lower limbs 32 frequent (33%) HP:0010741
30 polyneuropathy 32 occasional (7.5%) HP:0001271
31 abnormality of chromosome segregation 32 frequent (33%) HP:0002916
32 upper airway obstruction 32 occasional (7.5%) HP:0002781
33 pleural effusion 32 occasional (7.5%) HP:0002202
34 orthostatic hypotension due to autonomic dysfunction 32 occasional (7.5%) HP:0004926
35 interstitial pulmonary abnormality 32 frequent (33%) HP:0006530
36 adrenal insufficiency 32 occasional (7.5%) HP:0000846
37 skin nodule 32 occasional (7.5%) HP:0200036
38 abnormal ekg 32 frequent (33%) HP:0003115
39 elevated alkaline phosphatase 32 frequent (33%) HP:0003155
40 chronic constipation 32 frequent (33%) HP:0012450
41 intermittent claudication 32 occasional (7.5%) HP:0004417
42 bradycardia 32 occasional (7.5%) HP:0001662
43 monoclonal immunoglobulin m proteinemia 32 frequent (33%) HP:0005508
44 multiple myeloma 32 frequent (33%) HP:0006775
45 autonomic bladder dysfunction 32 occasional (7.5%) HP:0005341
46 pulmonary edema 32 frequent (33%) HP:0100598
47 arthropathy 32 frequent (33%) HP:0003040
48 abnormality of the submandibular glands 32 occasional (7.5%) HP:0010287
49 mechanical ileus 32 occasional (7.5%) HP:0010676
50 plasmacytoma 32 occasional (7.5%) HP:0011857

MGI Mouse Phenotypes related to Al Amyloidosis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.55 B2M CTSK LYZ TTR VWF
2 hematopoietic system MP:0005397 9.35 B2M CTSK IL1RL1 LYZ VWF
3 immune system MP:0005387 9.02 B2M CTSK IL1RL1 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 262)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ketamine Approved, Vet_approved Phase 3 6740-88-1 3821
2
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3,Phase 1,Phase 2,Not Applicable 1177-87-3
3
Lenograstim Approved, Investigational Phase 3,Phase 2,Phase 1 135968-09-1
4
Melphalan Approved Phase 3,Phase 2,Phase 1,Not Applicable 148-82-3 4053 460612
5
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 1,Phase 2,Not Applicable 50-02-2 5743
6
Cyclophosphamide Approved, Investigational Phase 3,Phase 1,Phase 2,Not Applicable 6055-19-2, 50-18-0 2907
7
Lenalidomide Approved Phase 3,Phase 1,Phase 2,Not Applicable 191732-72-6 216326
8
Bortezomib Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable 179324-69-7 387447 93860
9
Mechlorethamine Approved, Investigational Phase 3,Phase 1,Phase 2,Not Applicable 51-75-2 4033
10
Iron Approved Phase 3,Phase 1,Phase 2 7439-89-6 23925
11
Darbepoetin alfa Approved, Investigational Phase 3,Phase 2 11096-26-7, 209810-58-2
12
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
13
Heparin Approved, Investigational Phase 3 9005-49-6 772 46507594
14
Dalteparin Approved Phase 3 9005-49-6
15
Ondansetron Approved Phase 3 99614-02-5 4595
16
Caspofungin Approved Phase 3,Phase 2 179463-17-3, 162808-62-0 468682 2826718
17
Amphotericin B Approved, Investigational Phase 3 1397-89-3 5280965 14956
18
Miconazole Approved, Investigational, Vet_approved Phase 3,Phase 1,Phase 2,Not Applicable 22916-47-8 4189
19
Guaifenesin Approved, Investigational, Vet_approved Phase 3,Not Applicable 93-14-1 3516
20
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
21
Morphine Approved, Investigational Phase 3,Not Applicable 57-27-2 5288826
22 Ixazomib Approved, Investigational Phase 3,Phase 1,Phase 2 1072833-77-2
23
Doxycycline Approved, Investigational, Vet_approved Phase 2, Phase 3,Not Applicable 564-25-0 54671203
24
Daratumumab Approved Phase 3,Phase 1,Phase 2 945721-28-8
25
Ginseng Approved, Investigational, Nutraceutical Phase 3 50647-08-0
26
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
27
Glycine Approved, Nutraceutical, Vet_approved Phase 3,Phase 1,Phase 2 56-40-6 750
28 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
29 Anesthetics, Dissociative Phase 3
30 Anesthetics, General Phase 3
31 Excitatory Amino Acid Antagonists Phase 3
32 Excitatory Amino Acids Phase 3
33 Central Nervous System Depressants Phase 3,Phase 1,Phase 2,Not Applicable
34 Analgesics Phase 3,Phase 2,Not Applicable
35 Anesthetics, Intravenous Phase 3
36 Neurotransmitter Agents Phase 3,Phase 1,Phase 2
37 Anesthetics Phase 3
38 Peripheral Nervous System Agents Phase 3,Phase 1,Phase 2,Not Applicable
39
protease inhibitors Phase 3,Phase 1,Phase 2,Not Applicable
40 Antineoplastic Agents, Alkylating Phase 3,Phase 1,Phase 2,Not Applicable
41 Alkylating Agents Phase 3,Phase 1,Phase 2,Not Applicable
42 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 1,Phase 2,Not Applicable
43 Immunologic Factors Phase 3,Phase 1,Phase 2,Not Applicable
44 Hormone Antagonists Phase 3,Phase 1,Phase 2,Not Applicable
45 glucocorticoids Phase 3,Phase 1,Phase 2,Not Applicable
46 Gastrointestinal Agents Phase 3,Phase 1,Phase 2,Not Applicable
47 Immunosuppressive Agents Phase 3,Phase 1,Phase 2,Not Applicable
48 Antineoplastic Agents, Hormonal Phase 3,Phase 1,Phase 2,Not Applicable
49 Hormones Phase 3,Phase 1,Phase 2,Not Applicable
50 Autonomic Agents Phase 3,Phase 1,Phase 2,Not Applicable

Interventional clinical trials:

(show top 50) (show all 169)
# 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 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
22 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
23 A Trial of Doxycycline vs. Standard Supportive Therapy in Newly-diagnosed Cardiac AL Amyloidosis Patients Undergoing Bortezomib-based Therapy Not yet recruiting NCT03474458 Phase 2, Phase 3 Doxycycline;Standard of care therapy
24 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
25 Trial of High Dose Melphalan/Stem Cell Transplant With or Without Bortezomib Terminated NCT02489500 Phase 3 Bortezomib;Melphalan;Neupogen
26 The VITAL Amyloidosis Study, a Global Phase 3, Efficacy and Safety Study of NEOD001 in Patients With AL Amyloidosis Terminated NCT02312206 Phase 3 NEOD001
27 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
28 Lenalidomide, Dexamethasone and Cyclophosphamide in Amyloidosis (AL) Unknown status NCT00981708 Phase 1, Phase 2 Lenalidomide, Dexamethasone and Cyclophosphamide
29 Peripheral Stem Cell Transplantation in Treating Patients With Multiple Myeloma or Other B-cell Cancers Unknown status NCT00003163 Phase 2 cyclophosphamide;melphalan
30 Human Immune Globulin in Treating Patients With Primary Amyloidosis That is Causing Heart Dysfunction Completed NCT00547365 Phase 1, Phase 2
31 Pomalidomide and Dexamethasone (PDex) in AL Amyloidosis Completed NCT01510613 Phase 2 Pomalidomide and Dexamethasone
32 A Trial of Treatment With Lenalidomide-Melphalan-Dexamethasone in Patients With Primary (AL) Amyloidosis Completed NCT00883623 Phase 2 Lenalidomide
33 Melphalan, Lenalidomide, and Dexamethasone in Treating Patients With Primary Systemic Amyloidosis Completed NCT00679367 Phase 2 dexamethasone;lenalidomide;melphalan
34 CC-5013 With or Without Dexamethasone in Treating Patients With Primary Systemic Amyloidosis Completed NCT00091260 Phase 2 dexamethasone;lenalidomide
35 S9628 Dexamethasone Plus Interferon Alfa in Treating Patients With Primary Systemic Amyloidosis Completed NCT00002849 Phase 2 dexamethasone
36 Melphalan, Bortezomib, and Stem Cell Transplant in Treating Patients With Primary Systemic Amyloidosis Completed NCT00790647 Phase 2 bortezomib;melphalan
37 Bortezomib, Cyclophosphamide, and Dexamethasone in Treating Patients With Primary Systemic Light Chain Amyloidosis Completed NCT01072773 Phase 2 bortezomib;cyclophosphamide;dexamethasone
38 Efficacy of CC-5013 (Revlimid or Lenalidomide) in Patients With Primary Systemic Amyloidosis Completed NCT00166413 Phase 2 CC-5013
39 Cyclophosphamide, Lenalidomide and Dexamethasone (CLD) for Previously Treated Patients With AL Amyloidosis Completed NCT00607581 Phase 2 cyclophosphamide;lenalidomide;dexamethasone
40 4'-Iodo-4'-Deoxydoxorubicin in Treating Patients With Primary Systemic Amyloidosis Completed NCT00003853 Phase 2 4'-iodo-4'-deoxydoxorubicin
41 Lenalidomide, Cyclophosphamide, and Dexamethasone in Treating Patients With Primary Systemic Amyloidosis Completed NCT00564889 Phase 2 cyclophosphamide;dexamethasone;lenalidomide
42 Melphalan, Thalidomide, and Dexamethasone in Treating Patients With Newly Diagnosed, Previously Untreated Primary Systemic Amyloidosis Completed NCT00089167 Phase 2 dexamethasone;melphalan;thalidomide
43 High-Dose Melphalan Plus Peripheral Stem Cell Transplantation in Treating Patients With Primary Systemic Amyloidosis Completed NCT00003353 Phase 2 melphalan
44 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
45 Lendexal in Patients With Primary Systemic Amyloidosis (AL) Newly Diagnosed Completed NCT01194791 Phase 2 Lenalidomide;Cyclophosphamide;Dexamethasone
46 Phase 1/2, Open Label, Dose Escalation Study of NEOD001 in Subjects With Light Chain (AL) Amyloidosis Completed NCT01707264 Phase 1, Phase 2 NEOD001
47 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
48 Chemotherapy Plus Peripheral Stem Cell Transplant in Treating Patients Who Have Multiple Myeloma or Primary Systemic Amyloidosis Completed NCT00007995 Phase 2 busulfan;cyclophosphamide;melphalan
49 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
50 Risk-adapted Therapy for Primary Systemic (AL) Amyloidosis Completed NCT01527032 Phase 2 melphalan, thalidomide and dexamethasone

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

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MalaCards organs/tissues related to Al Amyloidosis:

41
Bone, Liver, Heart, Skin, Kidney, Bone Marrow, Testes
Sources

Publications for Al Amyloidosis

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Articles related to Al Amyloidosis:

(show top 50) (show all 984)
# Title Authors Year
1
Growth Differentiation Factor 15 Is a New Biomarker for Survival and Renal Outcomes in Light Chain (AL) Amyloidosis. ( 29386197 )
2018
2
A Rare Case of Systemic AL Amyloidosis with Muscle Involvement: A Misleading Diagnosis. ( 29651353 )
2018
3
Modified high-dose melphalan and autologous stem cell transplantation for AL amyloidosis: Experience in 334 patients. ( 29933072 )
2018
4
MRI feature tracking strain is prognostic for all-cause mortality in AL amyloidosis. ( 29733684 )
2018
5
Evaluation of a new continuous mononuclear cell collection procedure in a single transplant center cohort enriched for AL amyloidosis patients. ( 29731423 )
2018
6
[<sup>18</sup>F]Florbetaben PET-CT confirms AL amyloidosis in a patient with WaldenstrAPm's Macroglobulinemia. ( 29650640 )
2018
7
Usefulness of gastroduodenal biopsy in the differential diagnosis of systemic AH amyloidosis from systemic AL amyloidosis. ( 29660165 )
2018
8
Management of the elderly patient with AL amyloidosis. ( 29801808 )
2018
9
Venetoclax induced a complete response in a patient with AL amyloidosis plateaued on CyBorD. ( 29351984 )
2018
10
Coital Hemoptysis Due to Cardiac AL Amyloidosis. ( 29866287 )
2018
11
Dropped head syndrome with proximal myopathy revealing AL amyloidosis. ( 29653282 )
2018
12
Clinical characteristics and diagnosis of a rare case of systemic AL amyloidosis: a descriptive study. ( 29849940 )
2018
13
High-dose melphalan and stem cell transplantation in AL amyloidosis with elevated cardiac biomarkers. ( 29884854 )
2018
14
Novel Approaches for the Management of AL Amyloidosis. ( 29951831 )
2018
15
Atraumatic Splenic Rupture Due to Ectopic Extramedullary Hematopoiesis after Autologous Stem Cell Transplantation in a Patient with AL Amyloidosis. ( 29093392 )
2018
16
Epidemiology of AL amyloidosis: a real-world study using US claims data. ( 29748430 )
2018
17
Predictive value of the new renal response criteria in AL amyloidosis treated with high dose melphalan and stem cell transplantation. ( 29430701 )
2018
18
The use of serum free light chain dimerization patterns assist in the diagnosis of AL amyloidosis. ( 29767409 )
2018
19
Autologous stem cell transplant for AL amyloidosis patients aged 70 to 75. ( 29933071 )
2018
20
Elevation of Plasmin-I+2-plasmin Inhibitor Complexes in Patients with AL Amyloidosis. ( 29321425 )
2018
21
Presentation and outcome with second-line treatment in AL amyloidosis previously sensitive to nontransplant therapies. ( 29101236 )
2018
22
Daratumumab proves safe and highly effective in AL amyloidosis. ( 29938774 )
2018
23
Pathology and diagnosis of renal non-AL amyloidosis. ( 28828707 )
2018
24
Idelalisib plus rituximab is effective in systemic AL amyloidosis secondary to chronic lymphocytic leukaemia. ( 28971495 )
2018
25
Elevation of Plasmin-α2-plasmin Inhibitor Complex Predicts the Diagnosis of Systemic AL Amyloidosis in Patients with Monoclonal Protein. ( 29021469 )
2018
26
Once AL amyloidosis: not always AL amyloidosis. ( 29516761 )
2018
27
Digoxin use in systemic light-chain (AL) amyloidosis: contra-indicated or cautious use? ( 29529877 )
2018
28
Depth of organ response in AL amyloidosis is associated with improved survival: grading the organ response criteria. ( 29581546 )
2018
29
Light chain type predicts organ involvement and survival in AL amyloidosis patients receiving stem cell transplantation. ( 29615413 )
2018
30
Biomarkers in AL amyloidosis: is the summit in sight? ( 29622535 )
2018
31
The Effect and Safety of Bortezomib in the Treatment of AL Amyloidosis: A Systematic Review and Meta-Analysis. ( 29622862 )
2018
32
Evaluation of minimal residual disease using next-generation flow cytometry in patients with AL amyloidosis. ( 29795385 )
2018
33
Gastroparesis in a Patient with Gastric AL Amyloidosis. ( 30022922 )
2018
34
MR neurography biomarkers to characterize peripheral neuropathy in AL amyloidosis. ( 30030328 )
2018
35
Outcomes of patients with AL amyloidosis and low serum free light chain levels at diagnosis. ( 30032640 )
2018
36
Patient-reported distress is prevalent in systemic light chain (AL) amyloidosis but not determined by severity of disease. ( 30032653 )
2018
37
Daratumumab for the treatment of AL amyloidosis. ( 30033840 )
2018
38
AL amyloidosis presenting with limb girdle myopathy. ( 30042220 )
2018
39
High-dose melphalan and stem cell transplantation in systemic AL amyloidosis in the era of novel anti-plasma cell therapy: a comprehensive review. ( 30089901 )
2018
40
Treatment of AL amyloidosis with bendamustine: a study of 122 patients. ( 30108065 )
2018
41
Chronic intestinal pseudo-obstruction due to al amyloidosis: a case report and literature review. ( 30232705 )
2018
42
Turn off the Tap! The Need for Induction Therapy for AL Amyloidosis Before Transplantation. ( 30244106 )
2018
43
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 )
2018
44
Novel iatrogenic amyloidosis caused by peptide drug liraglutide: a clinical mimic of AL amyloidosis. ( 30262557 )
2018
45
Daratumumab-based therapy in patients with heavily-pretreated AL amyloidosis. ( 30267007 )
2018
46
AL amyloidosis: advances in diagnostics and treatment. ( 30299492 )
2018
47
Cytogenetic intraclonal heterogeneity of plasma cell dyscrasia in AL amyloidosis as compared with multiple myeloma. ( 30327369 )
2018
48
Development and validation of a survival staging system incorporating BNP in patients with light chain (AL) amyloidosis. ( 30333122 )
2018
49
Acquired factor VII deficiency causing severe bleeding disorder secondary to AL amyloidosis of the liver. ( 30344985 )
2018
50
Intraoral manifestation of systemic AL amyloidosis with unique microscopic presentation of intracellular amyloid deposition in striated muscles. ( 30351869 )
2018
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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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Pathways related to Al Amyloidosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Innate Immune System 66
Show member pathways
 12.69 B2M CTSK IL1RL1 LYZ TTR VWF
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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 space GO:0005615 9.62 B2M CTSK LYZ TTR
2 azurophil granule lumen GO:0035578 9.26 LYZ TTR
3 specific granule lumen GO:0035580 9.16 B2M LYZ
4 extracellular region GO:0005576 9.1 B2M CTSK IL1RL1 LYZ TTR VWF
5 tertiary granule lumen GO:1904724 8.96 B2M LYZ

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

# Name GO ID Score Top Affiliating Genes
1 neutrophil degranulation GO:0043312 9.33 B2M LYZ TTR
2 retina homeostasis GO:0001895 8.96 B2M LYZ
3 cellular protein metabolic process GO:0044267 8.8 B2M LYZ TTR

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

# Name GO ID Score Top Affiliating Genes
1 collagen binding GO:0005518 8.96 CTSK VWF
2 identical protein binding GO:0042802 8.92 B2M LYZ TTR VWF
Sources

Sources for Al Amyloidosis

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 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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