MCID: ALP005
MIFTS: 34

Alpha Chain Disease

Categories: Gastrointestinal diseases, Immune diseases

Aliases & Classifications for Alpha Chain Disease

MalaCards integrated aliases for Alpha Chain Disease:

Name: Alpha Chain Disease 12 15
Immunoproliferative Small Intestinal Disease 70
Seligmann's Disease 12

Classifications:



External Ids:

Disease Ontology 12 DOID:0060126
UMLS 70 C0021071

Summaries for Alpha Chain Disease

Disease Ontology : 12 A heavy chain disease that results from an overproduction of alpha antibodies (IgA).

MalaCards based summary : Alpha Chain Disease, also known as immunoproliferative small intestinal disease, is related to heavy chain disease and lymphosarcoma, and has symptoms including fever, abdominal pain and diarrhea. An important gene associated with Alpha Chain Disease is JCHAIN (Joining Chain Of Multimeric IgA And IgM), and among its related pathways/superpathways are Downstream signaling events of B Cell Receptor (BCR) and Immune response Fc epsilon RI pathway. Affiliated tissues include small intestine, bone marrow and lymph node, and related phenotypes are hematopoietic system and immune system

Related Diseases for Alpha Chain Disease

Diseases in the Mu Chain Disease family:

Alpha Chain Disease Delta Chain Disease

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

(show top 50) (show all 114)
# Related Disease Score Top Affiliating Genes
1 heavy chain disease 30.5 SUCO MYOM2 JCHAIN CD79A
2 lymphosarcoma 29.8 CD22 BCL6
3 plasmacytoma 29.6 CD79A CD5 CCND1
4 mu chain disease 10.3 SUCO JCHAIN
5 alpha-heavy chain disease 10.3
6 primary cutaneous marginal zone b-cell lymphoma 10.3 MALT1 BCL10
7 phaeohyphomycosis 10.2 MALT1 BCL10
8 solitary osseous plasmacytoma 10.2 MYOM2 CD79A
9 immunodeficiency 11 10.2 MALT1 BCL10
10 intestinal disease 10.2
11 nodal marginal zone b-cell lymphoma 10.2 MALT1 BCL6
12 hodgkin's lymphoma, lymphocytic-histiocytic predominance 10.2 JCHAIN BCL6
13 sclerosing hepatic carcinoma 10.1 CD79A CD5
14 monoclonal paraproteinemia 10.1 CD79A CD19
15 refractory hematologic cancer 10.1 CD22 CD19
16 refractory hairy cell leukemia 10.1 CD22 CD19
17 paranasal sinus lymphoma 10.1 CD79A BCL6
18 diarrhea 10.1
19 small intestine lymphoma 10.1
20 cerebral lymphoma 10.1 CD79A CD19
21 mediastinal cancer 10.1 CD79A CD5
22 b cell prolymphocytic leukemia 10.1 CD5 CD19
23 hodgkin's lymphoma, nodular sclerosis 10.1 CD79A BCL6
24 ureteral lymphoma 10.1 CD5 BCL6
25 thyrotoxic exophthalmos 10.1 CD5 CCND1
26 acute poststreptococcal glomerulonephritis 10.1 MYOM2 CD79A
27 prostate lymphoma 10.1 CD5 BCL6
28 gray zone lymphoma 10.1 CD79A BCL6
29 actinobacillosis 10.1 CD79A CD5
30 intravascular large b-cell lymphoma 10.0 CD5 BCL6
31 pediatric lymphoma 10.0 CD79A BCL6
32 immunoglobulin alpha deficiency 10.0 JCHAIN CD79A CD19
33 pleural empyema 10.0 CD79A BCL6
34 nodular lymphocyte predominant hodgkin lymphoma 10.0 CCND1 BCL6
35 nasal cavity lymphoma 10.0 CD5 BCL6
36 orbit lymphoma 10.0 MALT1 FOXP1 BIRC3 BCL10
37 central nervous system lymphoma 10.0 MALT1 FOXP1 BCL6
38 relapsed/refractory diffuse large b-cell lymphoma 10.0 FOXP1 CD22 CD19
39 testicular lymphoma 10.0 FOXP1 CD79A BCL6
40 extramedullary plasmacytoma 10.0 MYOM2 CD79A CCND1
41 plasma protein metabolism disease 10.0 MYOM2 CD5 CD19
42 gamma heavy chain disease 10.0
43 overgrowth syndrome 10.0
44 colon lymphoma 9.9 CD79A CD5 BCL6
45 capillary leak syndrome 9.9 CD22 CD19
46 duodenitis 9.9 MALT1 GAST
47 monoclonal gammopathy of uncertain significance 9.9 MYOM2 CD19 CCND1
48 isolated growth hormone deficiency 9.9 JCHAIN CD79A CD19
49 sezary's disease 9.9 CD5 BCL6 BCL10
50 mature t-cell and nk-cell lymphoma 9.9 CD79A CD5 BCL6

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



Diseases related to Alpha Chain Disease

Symptoms & Phenotypes for Alpha Chain Disease

UMLS symptoms related to Alpha Chain Disease:


fever; abdominal pain; diarrhea

MGI Mouse Phenotypes related to Alpha Chain Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.93 BCL10 BCL6 BIRC3 CCND1 CD19 CD22
2 immune system MP:0005387 9.77 BCL10 BCL6 BIRC3 CCND1 CD19 CD22
3 mortality/aging MP:0010768 9.36 BCL10 BCL6 BIRC3 CCND1 CD19 CD22

Drugs & Therapeutics for Alpha Chain Disease

Search Clinical Trials , NIH Clinical Center for Alpha Chain Disease

Genetic Tests for Alpha Chain Disease

Anatomical Context for Alpha Chain Disease

MalaCards organs/tissues related to Alpha Chain Disease:

40
Small Intestine, Bone Marrow, Lymph Node, Colon

Publications for Alpha Chain Disease

Articles related to Alpha Chain Disease:

(show top 50) (show all 198)
# Title Authors PMID Year
1
[Alpha chain disease: From bedside to bench and back again, an exemplary translation]. 61
30528152 2019
2
Alpha-chain disease: a lymphomagenesis model. 61
29976467 2018
3
[Intestinal non-Hodgkin lymphoma: "immunoproliferative small intestinal disease"]. 61
21458210 2011
4
[Algorithm for diagnosis of small intestinal diseases]. 61
18491580 2008
5
Immunoproliferative small intestinal disease in Greece: presentation of 13 cases including two from Albania. 61
16894320 2006
6
Immunoproliferative small intestinal disease associated with Campylobacter jejuni. 61
14724303 2004
7
[Etiological diagnosis of villous atrophy]. 61
11685130 2001
8
Gastrointestinal lymphoma: prevention and treatment of early lesions. 61
11355919 2001
9
Pathology of intestinal lymphomas. 61
10802861 2000
10
[Immunoproliferative disease of the small intestine. A case of polymeric IgA with evolution to lymphoma]. 61
9808897 1998
11
T-cell rich alpha-chain disease mimicking T-cell lymphoma. 61
9147095 1997
12
Non-secretory alpha chain disease involving stomach, small intestine and colon. 61
7606093 1995
13
[Gastrointestinal abnormalities in alpha-chain disease]. 61
7837478 1994
14
[Gastrointestinal abnormalities in alpha-chain disease]. 61
7736050 1994
15
[Heavy chain diseases]. 61
8502962 1993
16
Alpha-chain disease: analysis of alpha-chain protein and secretory component in jejunal fluid. 61
8419251 1993
17
Leukemic manifestation in a case of alpha-chain disease with multiple polypoid intestinal lymphocytic lymphoma. 61
1415195 1992
18
[Radiologic diagnosis of malignant lymphoma of the small intestine]. 61
1364781 1991
19
[Radiodiagnosis of malignant lymphoma of the small intestine]. 61
1877163 1991
20
A case of lymphoma-type alpha-chain disease. 61
1950370 1991
21
[Malt digestive system lymphomas and alpha heavy chain diseases. Histological and immunohistochemical study. Apropos of 3 cases]. 61
1905525 1991
22
Low serum transglutaminase in patients with intestinal lymphoma and alpha-chain disease. 61
1979203 1990
23
Gene deletions force nonsecretory alpha-chain disease plasma cells to produce membrane-form alpha-chain only. 61
2120331 1990
24
[Alpha heavy chain disease: a clinical study of 3 cases and review of the literature]. 61
2115016 1990
25
The value of double-contrast study of the small intestine in immunoproliferative small intestinal disease. 61
2318388 1990
26
Immunoproliferative small intestinal disease (IPSID): relationships with alpha-chain disease and "Mediterranean" lymphomas. 61
2205943 1990
27
[Clinico-roentgenological diagnosis of alpha chain disease]. 61
2123568 1990
28
[Local immunity in pathology of the IgA system (IgA subclasses in the saliva of patients with A-paraproteinemia)]. 61
2251665 1990
29
Nonsecretory alpha-chain disease with immunoproliferative small-intestinal disease. 61
2498659 1989
30
A case of alpha-chain disease. 61
2810318 1989
31
Treatment of alpha chain disease. Results of a prospective study in 21 Tunisian patients by the Tunisian-French intestinal Lymphoma Study Group. 61
2920354 1989
32
Immunoproliferative small intestinal disease without alpha-chain disease: a pathological study. 61
2914638 1989
33
Cells expressing intracytoplasmic immunoglobulins in secreting and hyposecreting cases of alpha chain disease. 61
2518741 1989
34
Lymphoma of the small intestine: radiological appearances. 61
3243056 1988
35
Cytopathology of alpha chain disease involving the central nervous system and pleura. 61
2462320 1988
36
A new form of alpha-chain disease with generalized lymph node involvement. 61
3222174 1988
37
Massive plasma cell infiltration of the digestive tract. Secretory component as the rate-limiting factor of immunoglobulin secretion in external fluids. 61
3410224 1988
38
Diffuse small intestinal lymphoid infiltration in nonimmunodeficient adults from Western Europe. 61
3292337 1988
39
Results of laparotomy in immunoproliferative small intestinal disease. 61
3349430 1988
40
[Morphologic changes of the small intestine and lymph nodes in alpha-chain disease]. 61
3143343 1988
41
Clinical and pathological evolution of alpha chain disease to immunoblastic lymphoma and response to COMP chemotherapy. 61
3352536 1988
42
Alpha-chain disease in children. 61
3335961 1988
43
Abnormal protein hyposecretion in early and late course of alpha chain disease. 61
3151749 1988
44
Affinity chromatography in the purification of alpha chain disease proteins. 61
3151750 1988
45
Immunoglobulin gene rearrangement in immunoproliferative small intestinal disease (IPSID). 61
3121678 1987
46
Immunoproliferative small-intestinal disease: clinical features and outcome in 30 cases. 61
3683173 1987
47
Alpha-chain disease. Report of a case from Taiwan. 61
3791162 1987
48
Malabsorption associated with nonmalignant immunoproliferative small intestinal disease. 61
3596077 1987
49
[Immunoblastic sarcoma localized in the peri-pancreatic region and appearing after a complete remission of alpha chain disease]. 61
3451440 1986
50
Gastric signet-ring cell lymphoma with alpha heavy chains. 61
3091474 1986

Variations for Alpha Chain Disease

Expression for Alpha Chain Disease

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

Pathways for Alpha Chain Disease

Pathways related to Alpha Chain Disease according to GeneCards Suite gene sharing:

(show all 11)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.36 MALT1 CD79A CD22 CD19 BCL10
2
Show member pathways
12.29 CD79A CD22 CD19 BCL6
3 12.17 MALT1 CD79A CD19 BCL6 BCL10
4
Show member pathways
11.98 MALT1 CD22 CD19 BCL6 BCL10
5 11.94 MALT1 BIRC3 BCL10
6
Show member pathways
11.63 CD79A CD22 CD19
7 11.6 MALT1 BIRC3 BCL10
8 11.54 CD5 CD22 CD19
9
Show member pathways
11.49 MALT1 CD79A CD22 CD19 BCL6 BCL10
10 11.42 SPN CD79A CD5 CD22 CD19
11 10.84 MALT1 BCL10

GO Terms for Alpha Chain Disease

Cellular components related to Alpha Chain Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 membrane raft GO:0045121 9.56 CD79A CD19 BIRC3 BCL10
2 external side of plasma membrane GO:0009897 9.35 SPN CD79A CD5 CD22 CD19
3 CBM complex GO:0032449 9.16 MALT1 BCL10
4 polkadots GO:0002096 8.62 MALT1 BCL10

Biological processes related to Alpha Chain Disease according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 adaptive immune response GO:0002250 9.83 JCHAIN CD79A CD19 BCL10
2 regulation of apoptotic process GO:0042981 9.73 MALT1 BIRC3 BCL6 BCL10
3 positive regulation of I-kappaB kinase/NF-kappaB signaling GO:0043123 9.71 MALT1 BIRC3 BCL10
4 regulation of inflammatory response GO:0050727 9.63 FOXP1 BIRC3 BCL6
5 regulation of immune response GO:0050776 9.62 SPN CD22 CD19 BCL6
6 positive regulation of protein ubiquitination GO:0031398 9.54 MALT1 BIRC3 BCL10
7 positive regulation of T cell activation GO:0050870 9.51 MALT1 BCL10
8 response to food GO:0032094 9.49 GAST BCL10
9 immunoglobulin mediated immune response GO:0016064 9.48 CD19 BCL10
10 negative regulation of B cell apoptotic process GO:0002903 9.4 FOXP1 BCL6
11 regulation of T cell receptor signaling pathway GO:0050856 9.26 MALT1 BCL10
12 response to fungus GO:0009620 9.16 MALT1 BCL10
13 B cell activation GO:0042113 9.13 MALT1 CD79A CD22
14 B-1 B cell differentiation GO:0001923 8.62 MALT1 CD19

Molecular functions related to Alpha Chain Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 kinase activator activity GO:0019209 8.96 MALT1 BCL10
2 protein self-association GO:0043621 8.8 MALT1 FOXP1 BCL10

Sources for Alpha Chain Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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