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

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Sources:
30ICD10, 31ICD10 via Orphanet, 54Orphanet, 68UMLS
See all MalaCards sources

Aliases & Descriptions for Alpha-Heavy Chain Disease:

Name: Alpha-Heavy Chain Disease 54
Immunoproliferative Small Intestinal Disease 54 68
Mediterranean Lymphoma 54
 
Alpha-Hcd 54
Ipsid 54

Characteristics:

Orphanet epidemiological data:

54
immunoproliferative small intestinal disease:
Age of onset: Adolescent,Adult; Age of death: adult

Classifications:



External Ids:

Orphanet54 ORPHA100025
ICD10 via Orphanet31 C88.3
ICD1030 C88.3

Summaries for Alpha-Heavy Chain Disease

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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 abdominal pain, diarrhea and fever. Affiliated tissues include small intestine and skin.

Related Diseases for Alpha-Heavy Chain Disease

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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:

idRelated DiseaseScoreTop Affiliating Genes
1alpha chain disease11.5
2heavy chain disease10.6
3lymphoma10.3
4intestinal disease10.1
5small intestine lymphoma9.8
6goiter9.8
7duodenal ulcer9.8
8duodenitis9.8
9gastrointestinal lymphoma9.8

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



Diseases related to alpha-heavy chain disease

Symptoms & Phenotypes for Alpha-Heavy Chain Disease

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Human phenotypes related to Alpha-Heavy Chain Disease:

 54 64 (show all 16)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 growth delay64 54 Occasional (29-5%) HP:0001510
2 ascites64 54 Occasional (29-5%) HP:0001541
3 alopecia64 54 Occasional (29-5%) HP:0001596
4 splenomegaly64 54 Occasional (29-5%) HP:0001744
5 anemia64 54 Frequent (79-30%) HP:0001903
6 fever64 54 Occasional (29-5%) HP:0001945
7 malabsorption64 54 Very frequent (99-80%) HP:0002024
8 abdominal pain64 54 Frequent (79-30%) HP:0002027
9 hepatomegaly64 54 Occasional (29-5%) HP:0002240
10 abnormality of the small intestine64 54 Very frequent (99-80%) HP:0002244
11 lymphoma64 54 Occasional (29-5%) HP:0002665
12 lymphadenopathy64 54 Occasional (29-5%) HP:0002716
13 immunodeficiency64 54 Frequent (79-30%) HP:0002721
14 hypocalcemia64 54 Frequent (79-30%) HP:0002901
15 dysgammaglobulinemia64 54 Very frequent (99-80%) HP:0002961
16 precocious menopause64 54 Occasional (29-5%) HP:0100805

UMLS symptoms related to Alpha-Heavy Chain Disease:


abdominal pain, diarrhea, fever

Drugs & Therapeutics for Alpha-Heavy Chain Disease

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Interventional clinical trials:

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

Genetic Tests for Alpha-Heavy Chain Disease

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Anatomical Context for Alpha-Heavy Chain Disease

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

36
Small intestine, Skin

Publications for Alpha-Heavy Chain Disease

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Articles related to Alpha-Heavy Chain Disease:

(show all 38)
idTitleAuthorsYear
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

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Expression for genes affiliated with Alpha-Heavy Chain Disease

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

Pathways for genes affiliated with Alpha-Heavy Chain Disease

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GO Terms for genes affiliated with Alpha-Heavy Chain Disease

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Sources for Alpha-Heavy Chain Disease

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet