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MCID: ALM001
MIFTS: 57

Al Amyloidosis

Categories: Rare diseases, Metabolic diseases, Nephrological diseases, Bone diseases, Immune diseases, Genetic diseases, Neuronal diseases, Cardiovascular diseases, Blood 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 Immune diseases Neuronal diseases Cardiovascular diseases Blood 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 hereditary amyloidosis. An important gene associated with Al Amyloidosis is LYZ (Lysozyme), and among its related pathways/superpathways are Innate Immune System and Cell adhesion_Cell-matrix glycoconjugates. The drugs Ketamine and Dexamethasone have been mentioned in the context of this disorder. Affiliated tissues include bone, bone marrow and heart, and related phenotypes are renal insufficiency and proteinuria

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 116)
# Related Disease Score Top Affiliating Genes
1 amyloidosis 30.5 LYZ TTR
2 hereditary amyloidosis 28.9 LYZ TTR
3 amyloidosis, primary localized cutaneous, 1 12.3
4 amyloidosis, primary localized cutaneous, 2 12.3
5 primary cutaneous amyloidosis 12.0
6 amyloidosis, primary localized cutaneous, 3 12.0
7 myeloma, multiple 11.6
8 light chain deposition disease 11.1
9 primary localized amyloidosis 11.1
10 amyloidosis, hereditary, transthyretin-related 10.8
11 hepatitis 10.1
12 factor x deficiency 10.1
13 nephrotic syndrome 10.0
14 lymphoma 10.0
15 venous insufficiency 9.9 MMP1 VWF
16 immune system disease 9.9 IL3 VWF
17 cholestasis 9.9
18 monoclonal gammopathy of uncertain significance 9.9
19 neuropathy 9.9
20 macroglobulinemia 9.9
21 depression 9.9
22 varicose veins 9.8 MMP1 VWF
23 macroglossia 9.8
24 infantile liver failure syndrome 1 9.8
25 glomerulonephritis 9.8
26 leukemia 9.8
27 mediastinitis 9.8
28 gastroduodenitis 9.8
29 endotheliitis 9.8
30 autoimmune disease 9.8
31 rheumatoid arthritis 9.8
32 aging 9.8
33 anxiety 9.8
34 autoimmune disease 1 9.8
35 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.8
36 autoimmune lymphoproliferative syndrome, type v 9.8
37 arthritis 9.8
38 b-cell lymphomas 9.8
39 dilated cardiomyopathy 9.8
40 colitis 9.8
41 atrial fibrillation 9.8
42 mucositis 9.8
43 acquired von willebrand syndrome 9.8
44 disseminated intravascular coagulation 9.8
45 polyneuropathy 9.8
46 hypothyroidism 9.8
47 intrahepatic cholestasis 9.8
48 hepatitis c 9.8
49 plasmacytoma 9.8
50 myopathy 9.8

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 54)
# Description HPO Frequency HPO Source Accession
1 renal insufficiency 32 frequent (33%) HP:0000083
2 proteinuria 32 frequent (33%) HP:0000093
3 nephrotic syndrome 32 frequent (33%) HP:0000100
4 enlarged kidney 32 frequent (33%) HP:0000105
5 nephropathy 32 frequent (33%) HP:0000112
6 macroglossia 32 occasional (7.5%) HP:0000158
7 xerostomia 32 occasional (7.5%) HP:0000217
8 hematuria 32 occasional (7.5%) HP:0000790
9 hypertension 32 occasional (7.5%) HP:0000822
10 adrenal insufficiency 32 occasional (7.5%) HP:0000846
11 goiter 32 occasional (7.5%) HP:0000853
12 purpura 32 occasional (7.5%) HP:0000979
13 keratoconjunctivitis sicca 32 occasional (7.5%) HP:0001097
14 polyneuropathy 32 occasional (7.5%) HP:0001271
15 congestive heart failure 32 frequent (33%) HP:0001635
16 hypertrophic cardiomyopathy 32 hallmark (90%) HP:0001639
17 bradycardia 32 occasional (7.5%) HP:0001662
18 asplenia 32 occasional (7.5%) HP:0001746
19 malabsorption 32 occasional (7.5%) HP:0002024
20 dyspnea 32 frequent (33%) HP:0002094
21 pleural effusion 32 occasional (7.5%) HP:0002202
22 gastrointestinal hemorrhage 32 occasional (7.5%) HP:0002239
23 hepatomegaly 32 frequent (33%) HP:0002240
24 autonomic dysregulation 32 occasional (7.5%) HP:0002271
25 aortic root aneurysm 32 occasional (7.5%) HP:0002616
26 lymphadenopathy 32 frequent (33%) HP:0002716
27 osteoarthritis 32 occasional (7.5%) HP:0002758
28 upper airway obstruction 32 occasional (7.5%) HP:0002781
29 arthralgia 32 frequent (33%) HP:0002829
30 abnormality of chromosome segregation 32 frequent (33%) HP:0002916
31 arthropathy 32 frequent (33%) HP:0003040
32 abnormal ekg 32 frequent (33%) HP:0003115
33 elevated alkaline phosphatase 32 frequent (33%) HP:0003155
34 decreased antibody level in blood 32 occasional (7.5%) HP:0004313
35 intermittent claudication 32 occasional (7.5%) HP:0004417
36 orthostatic hypotension due to autonomic dysfunction 32 occasional (7.5%) HP:0004926
37 autonomic bladder dysfunction 32 occasional (7.5%) HP:0005341
38 monoclonal immunoglobulin m proteinemia 32 frequent (33%) HP:0005508
39 abnormality of bone marrow cell morphology 32 occasional (7.5%) HP:0005561
40 interstitial pulmonary abnormality 32 frequent (33%) HP:0006530
41 multiple myeloma 32 frequent (33%) HP:0006775
42 abnormal blistering of the skin 32 occasional (7.5%) HP:0008066
43 abnormality of the submandibular glands 32 occasional (7.5%) HP:0010287
44 mechanical ileus 32 occasional (7.5%) HP:0010676
45 edema of the lower limbs 32 frequent (33%) HP:0010741
46 plasmacytoma 32 occasional (7.5%) HP:0011857
47 acute infectious pneumonia 32 frequent (33%) HP:0011949
48 hepatitis 32 occasional (7.5%) HP:0012115
49 fatigue 32 hallmark (90%) HP:0012378
50 chronic constipation 32 frequent (33%) HP:0012450
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 260)
# 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
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
Cyclophosphamide Approved, Investigational Phase 3,Phase 1,Phase 2,Not Applicable 50-18-0, 6055-19-2 2907
6
Lenalidomide Approved Phase 3,Phase 1,Phase 2,Not Applicable 191732-72-6 216326
7
Bortezomib Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable 179324-69-7 387447 93860
8
Mechlorethamine Approved, Investigational Phase 3,Phase 1,Phase 2,Not Applicable 51-75-2 4033
9
Darbepoetin alfa Approved, Investigational Phase 3,Phase 2 11096-26-7, 209810-58-2
10
Iron Approved Phase 3,Phase 1,Phase 2 7439-89-6 23925
11
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
12
Dalteparin Approved Phase 3 9005-49-6
13
Heparin Approved, Investigational Phase 3 9005-49-6 772 46507594
14
Ondansetron Approved Phase 3 99614-02-5 4595
15
Amphotericin B Approved, Investigational Phase 3 1397-89-3 14956 5280965
16
Caspofungin Approved Phase 3,Phase 2 179463-17-3, 162808-62-0 468682 2826718
17
Miconazole Approved, Investigational, Vet_approved Phase 3,Phase 1,Phase 2,Not Applicable 22916-47-8 4189
18
Dextromethorphan Approved Phase 3 125-71-3 5360696 5362449
19
Guaifenesin Approved, Investigational, Vet_approved Phase 3,Not Applicable 93-14-1 3516
20
Morphine Approved, Investigational Phase 3,Not Applicable 57-27-2 5288826
21
Daratumumab Approved Phase 3,Phase 1,Phase 2 945721-28-8
22 Ixazomib Approved, Investigational Phase 3,Phase 1,Phase 2 1072833-77-2
23
Ginseng Approved, Investigational, Nutraceutical Phase 3 50647-08-0
24
St. John's Wort Approved, Investigational, Nutraceutical Phase 3 84082-80-4
25
Glycine Approved, Nutraceutical, Vet_approved Phase 3,Phase 1,Phase 2 56-40-6 750
26 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
27 Analgesics Phase 3,Phase 2,Not Applicable
28 Anesthetics Phase 3
29 Anesthetics, Dissociative Phase 3
30 Anesthetics, General Phase 3
31 Anesthetics, Intravenous Phase 3
32 Central Nervous System Depressants Phase 3,Phase 1,Phase 2,Not Applicable
33 Excitatory Amino Acid Antagonists Phase 3
34 Excitatory Amino Acids Phase 3
35 Neurotransmitter Agents Phase 3,Phase 1,Phase 2
36 Peripheral Nervous System Agents Phase 3,Phase 1,Phase 2,Not Applicable
37 Alkylating Agents Phase 3,Phase 1,Phase 2,Not Applicable
38 Antiemetics Phase 3,Phase 1,Phase 2,Not Applicable
39 Anti-Inflammatory Agents Phase 3,Phase 1,Phase 2,Not Applicable
40 Antineoplastic Agents, Alkylating Phase 3,Phase 1,Phase 2,Not Applicable
41 Antineoplastic Agents, Hormonal Phase 3,Phase 1,Phase 2,Not Applicable
42 Autonomic Agents Phase 3,Phase 1,Phase 2,Not Applicable
43 BB 1101 Phase 3,Phase 1,Phase 2,Not Applicable
44 Dexamethasone acetate Phase 3,Phase 1,Phase 2,Not Applicable 1177-87-3
45 Gastrointestinal Agents Phase 3,Phase 1,Phase 2,Not Applicable
46 glucocorticoids Phase 3,Phase 1,Phase 2,Not Applicable
47 HIV Protease Inhibitors Phase 3,Phase 1,Phase 2,Not Applicable
48 Hormone Antagonists Phase 3,Phase 1,Phase 2,Not Applicable
49 Hormones Phase 3,Phase 1,Phase 2,Not Applicable
50 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 1,Phase 2,Not Applicable

Interventional clinical trials:

(show top 50) (show all 180)
# 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 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
23 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
24 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
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 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
47 Chemotherapy Plus Peripheral Stem Cell Transplant in Treating Patients Who Have Multiple Myeloma or Primary Systemic Amyloidosis Completed NCT00007995 Phase 2 busulfan;cyclophosphamide;melphalan
48 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
49 Risk-adapted Therapy for Primary Systemic (AL) Amyloidosis Completed NCT01527032 Phase 2 melphalan, thalidomide and dexamethasone
50 Autologous Stem Cell Rescue for Primary Amyloidosis Completed NCT00186407 Phase 2

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

Publications for Al Amyloidosis

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Articles related to Al Amyloidosis:

(show top 50) (show all 632)
# 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
Evaluation of minimal residual disease using next-generation flow cytometry in patients with AL amyloidosis. ( 29795385 )
2018
24
Prognostic significance of stringent complete response post stem cell transplantation in AL amyloidosis. ( 29964192 )
2018
25
Elevation of serum lactate dehydrogenase in AL amyloidosis reflects tissue damage and is an adverse prognostic marker in patients not eligible for stem cell transplantation. ( 28699650 )
2017
26
Comparison of Different Stem Cell Mobilization Regimens in AL Amyloidosis Patients. ( 28754546 )
2017
27
Attitudes about when and how to treat patients with AL amyloidosis: an international survey. ( 28857614 )
2017
28
Temporary Biventricular Assist Device to Bridge a Patient With Cardiogenic Shock Due to AL Amyloidosis to Heart Transplantation. ( 29230849 )
2017
29
Role of<sup>99m</sup>Tc-DPD scintigraphy in imaging extra-cardiac light chain (AL) amyloidosis. ( 29082513 )
2017
30
Congestive Heart Failure with Preserved Ejection Fraction in a Patient with Light Chain (AL) Amyloidosis and Multiple Myeloma. ( 28701686 )
2017
31
Real world data of the impact of first cycle daratumumab on multiple myeloma and AL amyloidosis services. ( 28832959 )
2017
32
The prognostic value of multiparametric flow cytometry in AL amyloidosis at diagnosis and at the end of first-line treatment. ( 27729322 )
2017
33
AL amyloidosis: from molecular mechanisms to targeted therapies. ( 29222231 )
2017
34
Light Chain (AL) Amyloidosis: The Journey to Diagnosis. ( 28808991 )
2017
35
Unravelling hypothyroidism in AL amyloidosis: Authors' reply. ( 28861923 )
2017
36
Delineation of the timing of second-line therapy post-autologous stem cell transplant in patients with AL amyloidosis. ( 28807981 )
2017
37
Novel pathologic scoring tools predict end-stage kidney disease in light chain (AL) amyloidosis. ( 28758811 )
2017
38
Recurrent diffuse gastric bleeding as a leading symptom ofA gastrointestinal AL amyloidosis. ( 29212103 )
2017
39
Correction: Utility of abdominal skin plus subcutaneous fat and rectal mucosal biopsy in the diagnosis of AL amyloidosis with renal involvement. ( 29107944 )
2017
40
Rationale and design of DUAL study: Doxycycline to Upgrade response in light chain (AL) amyloidosis (DUAL): A phase 2 pilot study of a two-pronged approach of prolonged doxycycline with plasma cell-directed therapy in the treatment of AL amyloidosis. ( 29696194 )
2017
41
News in AL Amyloidosis ASH 2016: AA rapidly evolving field of investigation. ( 28725274 )
2017
42
Massive Upper and Lower GI Bleed from Simultaneous Primary (AL) Amyloidosis of the Stomach and Transverse Colon in a Patient with Multiple Myeloma. ( 29282382 )
2017
43
Lung adenocarcinoma expressing receptor for advanced glycation end-products with primary systemic AL amyloidosis: a case report and literature review. ( 28056871 )
2017
44
Neuralgic amyotrophy following high-dose melphalan and autologous peripheral blood stem cell transplantation for AL amyloidosis. ( 29269794 )
2017
45
Utility of abdominal skin plus subcutaneous fat and rectal mucosal biopsy in the diagnosis of AL amyloidosis with renal involvement. ( 28926601 )
2017
46
Echocardiography as the first diagnostic clue to rapidly progressive systemic AL amyloidosis associated with multiple site thrombosis. A case report. ( 29197924 )
2017
47
Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis - An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS). ( 29176541 )
2017
48
Budd Chiari syndrome associated with AL amyloidosis: a coagulation paradox. ( 29219632 )
2017
49
The long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency. ( 29093408 )
2017
50
The incidence of atrial fibrillation among patients with AL amyloidosis undergoing high-dose melphalan and stem cell transplantation: experience at a single institution. ( 28737773 )
2017
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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 IL3 LGALS3 LYZ MMP1 TTR VWF
2
Cell adhesion_Cell-matrix glycoconjugates 22
10.55 LGALS3 MMP1
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 space GO:0005615 9.56 IL3 LGALS3 LYZ TTR
2 azurophil granule lumen GO:0035578 9.16 LYZ TTR
3 extracellular matrix GO:0031012 9.13 LGALS3 MMP1 VWF
4 extracellular region GO:0005576 9.1 IL3 LGALS3 LYZ MMP1 TTR VWF

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

# Name GO ID Score Top Affiliating Genes
1 neutrophil degranulation GO:0043312 9.13 LGALS3 LYZ TTR
2 cellular protein metabolic process GO:0044267 8.8 LYZ MMP1 TTR
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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