IPSID
MCID: ALP087
MIFTS: 23

Alpha-Heavy Chain Disease (IPSID) malady

Categories: Cancer diseases, Blood diseases, Rare diseases, Gastrointestinal diseases, Immune diseases

Aliases & Classifications for Alpha-Heavy Chain Disease

Aliases & Descriptions for Alpha-Heavy Chain Disease:

Name: Alpha-Heavy Chain Disease 56
Immunoproliferative Small Intestinal Disease 56 69
Mediterranean Lymphoma 56
Alpha-Hcd 56
Ipsid 56

Characteristics:

Orphanet epidemiological data:

56
alpha-heavy chain disease
Age of onset: Adolescent,Adult; Age of death: adult;

Classifications:



External Ids:

Orphanet 56 ORPHA100025
ICD10 via Orphanet 34 C88.3
ICD10 33 C88.3

Summaries for Alpha-Heavy Chain Disease

MalaCards based summary : Alpha-Heavy Chain Disease, also known as immunoproliferative small intestinal disease, is related to alpha chain disease and heavy chain disease, and has symptoms including growth delay, ascites and alopecia. Affiliated tissues include small intestine and skin.

Related Diseases for Alpha-Heavy Chain Disease

Diseases in the Heavy Chain Disease family:

Gamma Heavy Chain Disease Alpha-Heavy Chain Disease

Diseases related to Alpha-Heavy Chain Disease via text searches within MalaCards or GeneCards Suite gene sharing:

id Related Disease Score Top Affiliating Genes
1 alpha chain disease 11.5
2 heavy chain disease 10.6
3 lymphoma 10.3
4 intestinal disease 10.1
5 small intestine lymphoma 9.8
6 goiter 9.8
7 duodenal ulcer 9.8
8 duodenitis 9.8
9 gastrointestinal lymphoma 9.8

Graphical network of the top 20 diseases related to Alpha-Heavy Chain Disease:



Diseases related to Alpha-Heavy Chain Disease

Symptoms & Phenotypes for Alpha-Heavy Chain Disease

Human phenotypes related to Alpha-Heavy Chain Disease:

56 32 (show all 16)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 growth delay 56 32 Occasional (29-5%) HP:0001510
2 ascites 56 32 Occasional (29-5%) HP:0001541
3 alopecia 56 32 Occasional (29-5%) HP:0001596
4 splenomegaly 56 32 Occasional (29-5%) HP:0001744
5 anemia 56 32 Frequent (79-30%) HP:0001903
6 fever 56 32 Occasional (29-5%) HP:0001945
7 malabsorption 56 32 Very frequent (99-80%) HP:0002024
8 abdominal pain 56 32 Frequent (79-30%) HP:0002027
9 hepatomegaly 56 32 Occasional (29-5%) HP:0002240
10 abnormality of the small intestine 56 32 Very frequent (99-80%) HP:0002244
11 lymphoma 56 32 Occasional (29-5%) HP:0002665
12 lymphadenopathy 56 32 Occasional (29-5%) HP:0002716
13 immunodeficiency 56 32 Frequent (79-30%) HP:0002721
14 hypocalcemia 56 32 Frequent (79-30%) HP:0002901
15 dysgammaglobulinemia 56 32 Very frequent (99-80%) HP:0002961
16 precocious menopause 56 32 Occasional (29-5%) HP:0100805

UMLS symptoms related to Alpha-Heavy Chain Disease:


abdominal pain, diarrhea, fever

Drugs & Therapeutics for Alpha-Heavy Chain Disease

Search Clinical Trials , NIH Clinical Center for Alpha-Heavy Chain Disease

Genetic Tests for Alpha-Heavy Chain Disease

Anatomical Context for Alpha-Heavy Chain Disease

MalaCards organs/tissues related to Alpha-Heavy Chain Disease:

39
Small Intestine, Skin

Publications for Alpha-Heavy Chain Disease

Articles related to Alpha-Heavy Chain Disease:

(show all 38)
id Title Authors Year
1
Alpha heavy chain disease: a rare lymphoma hard to diagnose. ( 25125965 )
2014
2
Alpha heavy chain disease in a child. ( 10881592 )
2000
3
Alpha-heavy chain disease, Mediterranean lymphoma, and immunoproliferative small intestinal disease: a review of clinicopathological features, pathogenesis, and differential diagnosis. ( 10235185 )
1999
4
Failure of anti-lambda immunofixation reagent mimics alpha heavy-chain disease. ( 7813064 )
1995
5
Malabsorption associated with a high-grade-malignant non-Hodgkin's lymphoma, alpha-heavy-chain disease and immunoproliferative small intestinal disease. ( 7793120 )
1995
6
Immunoproliferative small intestinal disease: Mediterranean lymphoma and alpha heavy chain disease. ( 8313111 )
1994
7
Alpha heavy chain disease alpha mRNA contain nucleotide sequences of unknown origins. ( 1425927 )
1992
8
Immunoproliferative small intestinal disease: a study of 13 cases with alpha heavy-chain disease. ( 2227833 )
1990
9
Alpha heavy chain disease of patient MAL: structure of the non-functional rearranged alpha gene translocated on chromosome 9. ( 2125275 )
1990
10
Genomic alterations in a case of alpha heavy chain disease leading to the generation of composite exons from the JH region. ( 2513209 )
1989
11
Molecular analysis of a t(9;14)(p11;q32) translocation occurring in a case of human alpha heavy chain disease. ( 2498807 )
1989
12
Elevated serum IgA associated with immunoproliferative enteropathy of Basenji dogs: lack of evidence for alpha heavy-chain disease or enhanced intestinal IgA secretion. ( 3149068 )
1988
13
Severe malnutrition associated with alpha-heavy chain disease: response to tetracycline and intensive nutritional support. ( 3414653 )
1988
14
Gastric alpha heavy chain disease. ( 3120071 )
1987
15
Alpha-heavy-chain disease with erythematous skin lesions. ( 3096219 )
1986
16
Alpha-heavy chain disease. ( 3091314 )
1986
17
Alpha heavy chain disease: report of two cases. ( 3937780 )
1985
18
Plasma cell infiltration of the small bowel: lack of evidence for a non-secretory form of alpha-heavy chain disease. ( 3928450 )
1985
19
Two cases of alpha heavy chain disease in Venezuelans. ( 6442911 )
1984
20
Alpha heavy-chain disease presenting as goiter. ( 6431799 )
1984
21
Alpha heavy chain disease developing in an Asian resident in the United Kingdom and responding to antibiotics. ( 6413961 )
1983
22
Alpha-heavy chain disease. Report of a case with spontaneous regression. ( 6412354 )
1983
23
Alpha heavy chain disease lacking secretory alpha chain, with cobblestone appearance of the small intestine and duodenal ulcer demonstrated by endoscopy. ( 6804314 )
1982
24
Two alpha heavy chain disease proteins with different genomic deletions demonstrate that nonexpressed alpha heavy chain genes contain methylated bases. ( 6273910 )
1981
25
Follicular centre cell lymphoma with alpha heavy chain disease. A histopathological and immunohistological study. ( 6801847 )
1981
26
Abnormal chromosomal marker (D14 q+) in a patient with alpha heavy chain disease. ( 6767755 )
1980
27
Characterization of an antiserum specific for the Fabalpha fragment. Its use for detection of alpha-heavy chain disease protein by immunoselection. ( 762416 )
1979
28
The immunoselection technic in laboratory diagnosis of alpha heavy-chain disease. ( 110138 )
1979
29
Primary small intestinal lymphomas and alpha-heavy-chain disease. A study of 43 cases from a pathology department in Israel. ( 112083 )
1979
30
New presentation of alpha heavy chain disease: North American polypoid gastrointestinal lymphoma. Clinical and cellular studies. ( 416900 )
1978
31
Alpha heavy chain disease (report of 18 cases from Iraq). ( 98395 )
1978
32
Alpha heavy-chain disease in southern Iran. ( 411371 )
1977
33
The mutual clonal origin of the lymphoplasmocytic and lymphoma cell in alpha-heavy chain disease. ( 405165 )
1977
34
The evolution of alpha heavy chain disease. ( 818898 )
1976
35
Alpha heavy chain disease. ( 1148399 )
1975
36
Abdominal lymphoma with alpha-heavy chain disease. ( 809383 )
1975
37
Primary intestinal lymphoma and its relation to alpha heavy chain disease. ( 810150 )
1975
38
Alpha heavy chain disease. A variant of Mediterranean lymphoma. ( 5093214 )
1971

Variations for Alpha-Heavy Chain Disease

Expression for Alpha-Heavy Chain Disease

Search GEO for disease gene expression data for Alpha-Heavy Chain Disease.

Pathways for Alpha-Heavy Chain Disease

GO Terms for Alpha-Heavy Chain Disease

Sources for Alpha-Heavy Chain Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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