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IPSID
MCID: ALP087
MIFTS: 28

Alpha-Heavy Chain Disease (IPSID)

Categories: Blood diseases, Gastrointestinal diseases, Immune diseases, Rare diseases
Tissues Related diseases Publications Symptoms & Phenotypes
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Aliases & Classifications for Alpha-Heavy Chain Disease

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MalaCards integrated aliases for Alpha-Heavy Chain Disease:

Name: Alpha-Heavy Chain Disease 58
Immunoproliferative Small Intestinal Disease 58 17 71
Mediterranean Lymphoma 58
Alpha-Hcd 58
Ipsid 58

Characteristics:

Orphanet epidemiological data:

58
alpha-heavy chain disease
Age of onset: Adolescent,Adult; Age of death: adult;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Blood diseases Gastrointestinal diseases Immune diseases
See all MalaCards categories (disease lists)
ICD10: 33
Orphanet: 58  
Rare haematological diseases


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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 alpha chain disease, and has symptoms including fever, abdominal pain and diarrhea. Affiliated tissues include small intestine, b cells and bone, and related phenotypes are malabsorption and dysgammaglobulinemia

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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:

(show top 50) (show all 62)
# Related Disease Score Top Affiliating Genes
1 heavy chain disease 10.8
2 alpha chain disease 10.5
3 lymphoma 10.4
4 diarrhea 10.4
5 intestinal disease 10.4
6 b-cell lymphoma 10.3
7 marginal zone b-cell lymphoma 10.2
8 gastritis 10.2
9 small intestine lymphoma 10.2
10 diffuse large b-cell lymphoma 10.1
11 lymphoplasmacytic lymphoma 10.1
12 intestinal tuberculosis 10.1
13 peritonitis 10.1
14 lymphoma, mucosa-associated lymphoid type 10.1
15 gastrointestinal lymphoma 10.1
16 pelger-huet anomaly 10.1
17 dowling-degos disease 1 10.1
18 celiac disease 1 10.1
19 enterocolitis 10.1
20 lymphoma, hodgkin, classic 10.1
21 helicobacter pylori infection 10.1
22 lymphoma, non-hodgkin, familial 10.1
23 lymphoproliferative syndrome 10.1
24 tropical sprue 10.1
25 sarcoma 10.1
26 trichuriasis 10.1
27 hypothyroidism 10.1
28 keratosis 10.1
29 agammaglobulinemia 10.1
30 spindle cell sarcoma 10.1
31 hypokalemia 10.1
32 acquired immunodeficiency syndrome 10.1
33 alopecia 10.1
34 acute non lymphoblastic leukemia 10.1
35 burkitt lymphoma 9.9
36 gamma heavy chain disease 9.9
37 protein-losing enteropathy 9.9
38 goiter 9.9
39 duodenal ulcer 9.9
40 hepatitis b 9.9
41 extramedullary plasmacytoma 9.9
42 plasmacytoma 9.9
43 lymph node tuberculosis 9.9
44 pediatric lymphoma 9.9
45 intestinal obstruction 9.9
46 ileus 9.9
47 crohn's disease 9.9
48 lymphosarcoma 9.9
49 overgrowth syndrome 9.9
50 mycosis fungoides 9.8

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



Diseases related to Alpha-Heavy Chain Disease
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Symptoms & Phenotypes for Alpha-Heavy Chain Disease

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Human phenotypes related to Alpha-Heavy Chain Disease:

58 31 (show all 17)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 malabsorption 58 31 hallmark (90%) Very frequent (99-80%) HP:0002024
2 dysgammaglobulinemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002961
3 abnormality of the small intestine 58 31 hallmark (90%) Very frequent (99-80%) HP:0002244
4 immunodeficiency 58 31 frequent (33%) Frequent (79-30%) HP:0002721
5 anemia 58 31 frequent (33%) Frequent (79-30%) HP:0001903
6 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
7 hypocalcemia 58 31 frequent (33%) Frequent (79-30%) HP:0002901
8 splenomegaly 58 31 occasional (7.5%) Occasional (29-5%) HP:0001744
9 hepatomegaly 58 31 occasional (7.5%) Occasional (29-5%) HP:0002240
10 fever 58 31 occasional (7.5%) Occasional (29-5%) HP:0001945
11 ascites 58 31 occasional (7.5%) Occasional (29-5%) HP:0001541
12 growth delay 58 31 occasional (7.5%) Occasional (29-5%) HP:0001510
13 alopecia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001596
14 lymphoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0002665
15 lymphadenopathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0002716
16 premature ovarian insufficiency 31 occasional (7.5%) HP:0008209
17 precocious menopause 58 Occasional (29-5%)

UMLS symptoms related to Alpha-Heavy Chain Disease:


fever, abdominal pain, diarrhea
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Drugs & Therapeutics for Alpha-Heavy Chain Disease

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

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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:

40
Small Intestine, B Cells, Bone, Lymph Node, Colon, Skin, Testes
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Publications for Alpha-Heavy Chain Disease

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Articles related to Alpha-Heavy Chain Disease:

(show top 50) (show all 227)
# Title Authors PMID Year
1
Heavy Chain Disease of the Small Bowel. 61
29372346 2018
2
Recurrent diarrhoea in a renal transplant recipient: Early immunoproliferative small intestinal disease may be missed if not considered. 61
28205353 2017
3
Chronic diarrhea associated with high serum level of immunoglobulin A and diffuse infiltration of plasma cell in small intestine: A case report. 61
28151917 2017
4
Immunoproliferative Small Intestinal Disease Associated with Overwhelming Polymicrobial Gastrointestinal Infection with Transformation to Diffuse Large B-cell Lymphoma. 61
26903604 2016
5
Prevalence and Characteristics of Duodenal Villous Atrophy in Renal Transplant Patients Presenting With Persistent Diarrhea in a Developing Country. 61
26496241 2016
6
The role of infectious agents, antibiotics, and antiviral therapy in the treatment of extranodal marginal zone lymphoma and other low-grade lymphomas. 61
25975444 2015
7
Recent advances in intestinal lymphomas. 61
25639480 2015
8
Gastrointestinal complications in renal transplant recipients detected by endoscopic biopsies in a developing country. 61
25757628 2015
9
Immunoproliferative small intestinal disease: a report of 6 cases. 61
26349179 2014
10
Campylobacter coli cultured from the stools of a patient with immunoproliferative small intestinal disease. 61
24438451 2014
11
Primary gastrointestinal lymphomas - A study of 81 Cases from a Tertiary Healthcare Centre. 61
25494124 2014
12
Draft Genome Sequence of Campylobacter coli Strain IPSID-1 Isolated from a Patient with Immunoproliferative Small Intestinal Disease. 61
24625865 2014
13
MALT lymphoma of the small bowel with protein-losing enteropathy. 61
24395281 2014
14
Alpha heavy chain disease: a rare lymphoma hard to diagnose. 61
25125965 2014
15
Immunoproliferative small intestinal disease presented with ascites and edema. 61
24558774 2013
16
A case of mucosa-associated lymphoid tissue lymphoma of the gastrointestinal tract showing extensive plasma cell differentiation with prominent Russell bodies. 61
24303496 2013
17
Spectrum of malabsorption syndrome among adults & factors differentiating celiac disease & tropical malabsorption. 61
23041739 2012
18
Immunoproliferative small intestinal disease associated with Campylobacter jejuni. 61
22552252 2012
19
[Primary small intestinal lymphoma: epidemiological, histological and therapeutic transition in Tunisia]. 61
22450277 2012
20
A rare cause of lymphadenopathy near the terminal ileum: immunoproliferative small intestinal disease. 61
22505392 2012
21
Capsule-endoscopic findings in immunoproliferative small-intestinal disease. 61
22396282 2012
22
[Intestinal non-Hodgkin lymphoma: "immunoproliferative small intestinal disease"]. 61
21458210 2011
23
Intestinal lymphoma: a case report. 61
22195372 2011
24
Gastrointestinal lymphomas: pattern of distribution and histological subtypes: 10 years experience in a tertiary centre in South India. 61
22234096 2011
25
Spectrum of malabsorption in India--tropical sprue is still the leader. 61
22315745 2011
26
Immunoproliferative small intestinal disease-a rare extranodal marginal zone lymphoma of mucosa associated lymphoid tissue in the Indian subcontinent. 61
27365825 2011
27
Immunoproliferative small intestinal disease (IPSID). 61
21276391 2011
28
Regression of immunoproliferative small intestinal disease after eradication of Helicobacter pylori. 61
20300878 2010
29
Immunoproliferative small intestinal disease versus colonic monoblastic sarcoma in a 2-year-old boy. 61
19942975 2009
30
Coeliac disease. 61
19377424 2009
31
Immunoproliferative small intestinal disease with protein loss complicated with duodenal T cell lymphoma during progression. 61
18277034 2008
32
[Immunoproliferative small intestinal disease: a case report and literature review]. 61
17706052 2007
33
Emergency pancreatoduodenectomy (whipple procedure) for massive upper gastrointestinal bleeding caused by a diffuse B-cell lymphoma of the duodenum: report of a case. 61
17643214 2007
34
[Alpha heavy chain disease. A Tunisian case]. 61
17263208 2006
35
Immunoproliferative small intestinal disease in Greece: presentation of 13 cases including two from Albania. 61
16894320 2006
36
Sonoenteroclysis: a new technique for the diagnosis of small bowel diseases. 61
16447095 2006
37
Roles of syndecan-1, bcl6 and p53 in diagnosis and prognostication of immunoproliferative small intestinal disease. 61
16773719 2006
38
Infection-associated lymphomas derived from marginal zone B cells: a model of antigen-driven lymphoproliferation. 61
16397126 2006
39
Nonsecretory variant of immunoproliferative small intestinal disease: a case report with pathologic, immunophenotypic, and molecular findings. 61
16253033 2005
40
Immunoproliferative small intestinal disease: current concepts. 61
16259867 2005
41
Low-grade B-Cell lymphomas with plasmacytic differentiation lack PAX5 gene rearrangements. 61
16049306 2005
42
[Immunoproliferative small intestinal disease associated with Campylobacter jejuni]. 61
15978389 2005
43
Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms. 61
15542584 2005
44
[Immunoproliferative small intestinal disease associated with Campylobacter jejuni]. 61
15329812 2004
45
Immunoproliferative small intestinal disease associated with Campylobacter jejuni. 61
15084705 2004
46
[Malabsorption syndrome. Rare differential diagnosis in a young patient]. 61
14991163 2004
47
Immunoproliferative small intestinal disease associated with Campylobacter jejuni. 61
14724303 2004
48
Lymphomatous category of immunoproliferative small intestinal disease. 61
14616957 2003
49
Variable frequencies of t(11;18)(q21;q21) in MALT lymphomas of different sites: significant association with CagA strains of H pylori in gastric MALT lymphoma. 61
12676782 2003
50
Re: Lin and Gray - immunoproliferative small intestinal disease. 61
12591078 2003
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Variations for Alpha-Heavy Chain Disease

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Expression for Alpha-Heavy Chain Disease

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

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GO Terms for Alpha-Heavy Chain Disease

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Sources for Alpha-Heavy Chain Disease

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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
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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