MCID: ALP060
MIFTS: 26

Alpha Heavy-Chain Disease malady

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

Aliases & Classifications for Alpha Heavy-Chain Disease

About this section
Sources:
47Orphanet, 60UMLS, 26ICD10 via Orphanet, 25ICD10
See all sources

Alpha Heavy-Chain Disease, Aliases & Descriptions:

Name: Alpha Heavy-Chain Disease 47
Immunoproliferative Small Intestinal Disease 47 60
Mediterranean Lymphoma 47
 
Alpha-Hcd 47
Ipsid 47


Classifications:



Characteristics (Orphanet epidemiological data):

47
immunoproliferative small intestinal disease:
Age of onset: Adolescent,Adult; Age of death: adult


External Ids:

Orphanet47 100025
ICD10 via Orphanet26 C88.3
ICD1025 C88.3

Summaries for Alpha Heavy-Chain Disease

About this section


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.

Related Diseases for Alpha Heavy-Chain Disease

About this section

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:

(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

About this section

Symptoms:

 47 (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

About this section

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

About this section

Anatomical Context for Alpha Heavy-Chain Disease

About this section

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

31
Small intestine, Colon, Liver, Skin

Animal Models for Alpha Heavy-Chain Disease or affiliated genes

About this section

Publications for Alpha Heavy-Chain Disease

About this section

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

About this section

Expression for genes affiliated with Alpha Heavy-Chain Disease

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

Pathways for genes affiliated with Alpha Heavy-Chain Disease

About this section

Compounds for genes affiliated with Alpha Heavy-Chain Disease

About this section

GO Terms for genes affiliated with Alpha Heavy-Chain Disease

About this section

Products for genes affiliated with Alpha Heavy-Chain Disease

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies
  • Proteins
  • Kits and Assays

Sources for Alpha Heavy-Chain Disease

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet