MCID: ALP060
MIFTS: 30

Alpha Heavy-Chain Disease malady

Cancer diseases, Blood diseases, Rare diseases, Gastrointestinal diseases, Immune diseases, Oral diseases categories

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 heavy chain disease and intestinal disease, and has symptoms including weight loss, malabsorption and abnormal immunoglobulin level. Affiliated tissues include small intestine, colon and liver.

Aliases & Classifications for Alpha Heavy-Chain Disease

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Sources:
49Orphanet, 62UMLS, 28ICD10 via Orphanet, 27ICD10
See all sources

Alpha Heavy-Chain Disease, Aliases & Descriptions:

Name: Alpha Heavy-Chain Disease 49
Immunoproliferative Small Intestinal Disease 49 62
Ipsid 49 62
Alpha Heavy Chain Disease 62
 
Lymphoma, Mediterranean 62
Mediterranean Lymphoma 49
Alpha Hcd 62
Alpha-Hcd 49


Classifications:



Characteristics (Orphanet epidemiological data):

49
immunoproliferative small intestinal disease:
Age of onset: Adolescent,Adult; Age of death: adult


External Ids:

Orphanet49 100025
ICD10 via Orphanet28 C88.3
ICD1027 C88.3

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/GeneDecks gene sharing:

(show all 12)
idRelated DiseaseScoreTop Affiliating Genes
1heavy chain disease11.0
2intestinal disease10.5
3celiac disease10.3
4diarrhea10.3
5sarcoma10.3
6small intestine lymphoma10.2
7duodenal ulcer10.2
8gastrointestinal lymphoma10.2
9duodenitis10.2
10goiter10.2
11alpha chain disease10.1
12mature b-cell neoplasm10.1

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



Diseases related to alpha heavy-chain disease

Symptoms for Alpha Heavy-Chain Disease

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Symptoms:

 49 (show all 17)
  • intestinal/colonic anomaly
  • malabsorption/chronic diarrhea/steatorrhea
  • monoclonal immunoglobulins/gammapathy/dysglobulinemia
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • acute abdominal pain/colic
  • immunodeficiency/increased susceptibility to infections/recurrent infections
  • anaemia
  • hypocalcemia
  • alopecia
  • ascitis
  • hepatomegaly/liver enlargement (excluding storage disease)
  • splenomegaly
  • lymphadenopathy/polyadenopathies
  • precocious menopause/secondary amenorrhea
  • lymphoma
  • failure to thrive/difficulties for feeding in infancy/growth delay
  • fever/chilling

HPO human phenotypes related to Alpha Heavy-Chain Disease:

(show all 14)
id Description Frequency HPO Source Accession
1 weight loss hallmark (90%) HP:0001824
2 malabsorption hallmark (90%) HP:0002024
3 abnormal immunoglobulin level hallmark (90%) HP:0010701
4 anemia typical (50%) HP:0001903
5 abdominal pain typical (50%) HP:0002027
6 hypocalcemia typical (50%) HP:0002901
7 secondary amenorrhea occasional (7.5%) HP:0000869
8 ascites occasional (7.5%) HP:0001541
9 alopecia occasional (7.5%) HP:0001596
10 splenomegaly occasional (7.5%) HP:0001744
11 hepatomegaly occasional (7.5%) HP:0002240
12 lymphoma occasional (7.5%) HP:0002665
13 lymphadenopathy occasional (7.5%) HP:0002716
14 abnormality of temperature regulation occasional (7.5%) HP:0004370

Drugs & Therapeutics for Alpha Heavy-Chain Disease

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Drug clinical trials:

Search ClinicalTrials for Alpha Heavy-Chain Disease

Search 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:

33
Small intestine, Colon, Liver, Skin

Animal Models for Alpha Heavy-Chain Disease or affiliated genes

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Publications for Alpha Heavy-Chain Disease

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Articles related to Alpha Heavy-Chain Disease:

(show all 37)
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
Malabsorption associated with a high-grade-malignant non-Hodgkin's lymphoma, alpha-heavy-chain disease and immunoproliferative small intestinal disease. (7793120)
1995
5
Failure of anti-lambda immunofixation reagent mimics alpha heavy-chain disease. (7813064)
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. (3091314)
1986
16
Alpha-heavy-chain disease with erythematous skin lesions. (3096219)
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
Alpha heavy-chain disease presenting as goiter. (6431799)
1984
20
Two cases of alpha heavy chain disease in Venezuelans. (6442911)
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 lacking secretory alpha chain, with cobblestone appearance of the small intestine and duodenal ulcer demonstrated by endoscopy. (6804314)
1982
23
Two alpha heavy chain disease proteins with different genomic deletions demonstrate that nonexpressed alpha heavy chain genes contain methylated bases. (6273910)
1981
24
Follicular centre cell lymphoma with alpha heavy chain disease. A histopathological and immunohistological study. (6801847)
1981
25
Abnormal chromosomal marker (D14 q+) in a patient with alpha heavy chain disease. (6767755)
1980
26
Characterization of an antiserum specific for the Fabalpha fragment. Its use for detection of alpha-heavy chain disease protein by immunoselection. (762416)
1979
27
Primary small intestinal lymphomas and alpha-heavy-chain disease. A study of 43 cases from a pathology department in Israel. (112083)
1979
28
The immunoselection technic in laboratory diagnosis of alpha heavy-chain disease. (110138)
1979
29
Alpha heavy chain disease (report of 18 cases from Iraq). (98395)
1978
30
New presentation of alpha heavy chain disease: North American polypoid gastrointestinal lymphoma. Clinical and cellular studies. (416900)
1978
31
The mutual clonal origin of the lymphoplasmocytic and lymphoma cell in alpha-heavy chain disease. (405165)
1977
32
Alpha heavy-chain disease in southern Iran. (411371)
1977
33
The evolution of alpha heavy chain disease. (818898)
1976
34
Abdominal lymphoma with alpha-heavy chain disease. (809383)
1975
35
Primary intestinal lymphoma and its relation to alpha heavy chain disease. (810150)
1975
36
Alpha heavy chain disease. (1148399)
1975
37
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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Compounds 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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Products for genes affiliated with Alpha Heavy-Chain Disease

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies
  • Proteins
  • Kits and Assays

Sources for Alpha Heavy-Chain Disease

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4CDC
15ExPASy
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet