MCID: AHM002
MIFTS: 19

Ah Amyloidosis

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

Aliases & Classifications for Ah Amyloidosis

MalaCards integrated aliases for Ah Amyloidosis:

Name: Ah Amyloidosis 58
Heavy Chain Amyloidosis 58

Characteristics:

Orphanet epidemiological data:

58
ah amyloidosis
Inheritance: Not applicable;

Classifications:

Orphanet: 58  
Rare renal diseases
Rare systemic and rhumatological diseases


External Ids:

ICD10 via Orphanet 33 E85.9
Orphanet 58 ORPHA442582

Summaries for Ah Amyloidosis

MalaCards based summary : Ah Amyloidosis, also known as heavy chain amyloidosis, is related to amyloidosis and nephrotic syndrome. The drugs Altretamine and Vaccines have been mentioned in the context of this disorder. Affiliated tissues include t cells, bone and heart.

Related Diseases for Ah Amyloidosis

Diseases in the Amyloidosis family:

Al Amyloidosis Amyloidosis Aa
Hereditary Amyloidosis Primary Localized Amyloidosis
Ah Amyloidosis

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

(show all 14)
# Related Disease Score Top Affiliating Genes
1 amyloidosis 10.4
2 nephrotic syndrome 10.2
3 al amyloidosis 10.2
4 lymphoplasmacytic lymphoma 10.0
5 lymphoproliferative syndrome 10.0
6 plasma cell neoplasm 10.0
7 t-cell large granular lymphocyte leukemia 9.9
8 lymphocytic leukemia 9.9
9 corneal dystrophy 9.9
10 iga glomerulonephritis 9.9
11 eye disease 9.9
12 monoclonal gammopathy of uncertain significance 9.9
13 lattice corneal dystrophy 9.9
14 light chain deposition disease 9.9

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



Diseases related to Ah Amyloidosis

Symptoms & Phenotypes for Ah Amyloidosis

Drugs & Therapeutics for Ah Amyloidosis

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Altretamine Approved Early Phase 1 645-05-6 2123
2 Vaccines Early Phase 1
3 Plasma-lyte 148 Early Phase 1
4 Immunologic Factors Early Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Pilot Study of Dendritic Cell DKK1 Vaccine for Patients With Monoclonal Gammopathy and Stable or Smoldering Myeloma Not yet recruiting NCT03591614 Early Phase 1

Search NIH Clinical Center for Ah Amyloidosis

Genetic Tests for Ah Amyloidosis

Anatomical Context for Ah Amyloidosis

MalaCards organs/tissues related to Ah Amyloidosis:

40
T Cells, Bone, Heart, B Cells, Eye

Publications for Ah Amyloidosis

Articles related to Ah Amyloidosis:

(show all 27)
# Title Authors PMID Year
1
IgG╬║ light and heavy chain amyloidosis secondary to a B-cell lymphoproliferative disorder, whole picture on a renal biopsy. 61
31726478 2019
2
Underlying IgM heavy chain amyloidosis in treatment-refractory IgA nephropathy: A case report. 61
31624754 2019
3
Heavy Chain Fibrillary Glomerulonephritis: A Case Report. 61
30955945 2019
4
Monoclonal Immunoglobulin Deposition Disease and Related Diseases. 61
30918151 2019
5
A Case of T-Cell Large Granular Lymphocytic Leukemia and Renal Immunoglobulin Heavy Chain Amyloidosis. 61
30631033 2019
6
A case report - renal heavy chain amyloidosis and T-cell large granular lymphocytic leukaemia. 61
31343367 2019
7
Heavy-chain amyloidosis in gastroduodenal biopsies - the histopathological features. 61
31343299 2019
8
Usefulness of gastroduodenal biopsy in the differential diagnosis of systemic AH amyloidosis from systemic AL amyloidosis. 61
29660165 2018
9
Renal AH Amyloidosis Associated With a Truncated Immunoglobulin Heavy Chain Undetectable by Immunostaining. 61
26362695 2015
10
Bortezomib plus dexamethasone results in a late organ response in primary heavy-chain amyloidosis without a hematologic response. 61
25732670 2015
11
Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease. 61
25838650 2015
12
Rituximab-cyclophosphamide-dexamethasone is highly effective in patients with monoclonal Ig deposit-related glomerulopathy and indolent non-Hodgkin lymphomas. 61
25043930 2014
13
Proximal tubulopathies associated with monoclonal light chains: the spectrum of clinicopathologic manifestations and molecular pathogenesis. 61
25268200 2014
14
Non-light-chain immunoglobulin amyloidosis: time to expand or refine the spectrum to include light+heavy chain amyloidosis? 61
23446254 2013
15
The diagnosis and characteristics of renal heavy-chain and heavy/light-chain amyloidosis and their comparison with renal light-chain amyloidosis. 61
23302715 2013
16
Heavy-chain amyloidosis in TGFBI-negative and gelsolin-negative atypical lattice corneal dystrophy. 61
21743312 2011
17
Mass spectrometry-based proteomic diagnosis of renal immunoglobulin heavy chain amyloidosis. 61
20876678 2010
18
Rituximab therapy in nephrotic syndrome due to AH amyloidosis. 61
19590993 2009
19
AH amyloidosis associated with an immunoglobulin heavy chain variable region (VH1) fragment: a case report. 61
18484339 2008
20
Immunoglobulin light and heavy chain amyloidosis AL/AH: renal pathology and differential diagnosis. 61
17075228 2007
21
AH amyloidosis associated with lymphoplasmacytic lymphoma secreting a monoclonal gamma heavy chain carrying an unusual truncated D segment. 61
16632032 2006
22
Metachronous development of nonamyloidogenic [lambda] light chain deposition disease and IgG heavy chain amyloidosis in the same patient. 61
14576484 2003
23
Immunoglobulin heavy chain can be amyloidogenic: morphologic characterization including immunoelectron microscopy. 61
12657941 2003
24
Renal manifestations of plasma cell dyscrasias: an appraisal from the patients' bedside to the research laboratory. 61
10919389 2000
25
Primary amyloidosis associated with a novel heavy-chain fragment (AH amyloidosis). 61
8141123 1994
26
Immunoglobulin heavy-chain-associated amyloidosis. 61
2118650 1990
27
The pathogenesis and biochemistry of amyloidosis. 61
3550020 1987

Variations for Ah Amyloidosis

Expression for Ah Amyloidosis

Search GEO for disease gene expression data for Ah Amyloidosis.

Pathways for Ah Amyloidosis

GO Terms for Ah Amyloidosis

Sources for Ah Amyloidosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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