MCID: PLY010
MIFTS: 39

Polyclonal Hypergammaglobulinemia

Categories: Genetic diseases, Immune diseases, Metabolic diseases

Aliases & Classifications for Polyclonal Hypergammaglobulinemia

MalaCards integrated aliases for Polyclonal Hypergammaglobulinemia:

Name: Polyclonal Hypergammaglobulinemia 12 54 15 71

Classifications:



External Ids:

Disease Ontology 12 DOID:2344
ICD9CM 34 273.0
NCIt 49 C35885
ICD10 32 D89.0
UMLS 71 C0154254

Summaries for Polyclonal Hypergammaglobulinemia

MalaCards based summary : Polyclonal Hypergammaglobulinemia is related to cholangitis and multicentric castleman disease. An important gene associated with Polyclonal Hypergammaglobulinemia is IL6 (Interleukin 6), and among its related pathways/superpathways are Innate Immune System and Class I MHC mediated antigen processing and presentation. The drugs Immunologic Factors and Rho(D) Immune Globulin have been mentioned in the context of this disorder. Affiliated tissues include t cells, bone and b cells, and related phenotype is immune system.

Related Diseases for Polyclonal Hypergammaglobulinemia

Diseases related to Polyclonal Hypergammaglobulinemia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 497)
# Related Disease Score Top Affiliating Genes
1 cholangitis 30.5 IL6 CRP CD40LG
2 multicentric castleman disease 30.5 IL6 CRP CD4
3 lymphadenitis 30.4 SERPINA3 CRP CD4
4 mikulicz disease 30.3 CRP CD4
5 pericardial effusion 30.2 IL6 CRP CD40LG CD4
6 igg4-related disease 30.2 TNFSF13B CRP CD4
7 histiocytosis 30.1 SLC29A3 SERPINA3 CD1C CD1A
8 pneumonia 30.0 IL6 CRP CD8A CD40LG
9 cholangitis, primary sclerosing 29.9 IL6 CD8A CD40LG CD4
10 autoimmune disease 29.8 TRIM21 TNFSF13B IL6 CRP CD40LG
11 rheumatoid arthritis 29.5 TNFSF13B SERPINA3 IL6 CRP CD40LG CD4
12 lymphoma, mucosa-associated lymphoid type 29.5 TNFSF13B MME CD40LG CD4
13 pancytopenia 29.4 SERPINA3 IL6 CRP CD8A CD40LG CD4
14 exanthem 29.2 TRIM21 IL6 CRP CD8A CD40LG CD4
15 sjogren syndrome 29.1 TRIM21 TNFSF13B SSB CD40LG
16 leukemia, acute myeloid 28.9 SERPINA3 MME IL6 CD8A CD4
17 plasma cell neoplasm 28.8 SERPINA3 MYOM2 MME IL6 CD8A CD40LG
18 sialadenitis 28.5 TRIM21 TNFSF13B SSB CD8A CD4
19 myeloma, multiple 27.8 TNFSF13B MYOM2 MME IL6 CRP CD8A
20 lymphoid interstitial pneumonia 27.0 TRIM21 SSB IL6 CRP CD8A CD40LG
21 histiocytosis-lymphadenopathy plus syndrome 11.6
22 gaucher disease, type iii 11.5
23 intestinal tuberculosis 10.5 CRP CD4
24 cold agglutinin disease 10.5 IL6 CRP
25 prosthetic joint infection 10.5 IL6 CRP
26 cardiogenic shock 10.5 IL6 CRP
27 riedel's fibrosing thyroiditis 10.5 CD40LG CD4
28 cryopyrin-associated periodic syndrome 10.5 IL6 CRP
29 uremic pruritus 10.5 IL6 CRP
30 systemic autoimmune disease 10.5 IL6 CRP
31 orbital plasma cell granuloma 10.5 CRP CD40LG
32 chronic orbital inflammation 10.5 CRP CD40LG
33 japanese spotted fever 10.5 CRP CD40LG
34 latent syphilis 10.5 CD40LG CD4
35 gastroduodenitis 10.5 CD40LG CD4
36 cyclosporiasis 10.5 SERPINA3 CD4
37 primary syphilis 10.5 CD40LG CD4
38 asymptomatic neurosyphilis 10.5 CD40LG CD4
39 tertiary neurosyphilis 10.5 CD40LG CD4
40 transient arthritis 10.5 CD40LG CD4
41 intracranial sinus thrombosis 10.5 CRP CD40LG
42 splenic abscess 10.5 CRP CD40LG
43 pericardial tuberculosis 10.5 CRP CD4
44 meningovascular neurosyphilis 10.5 CD40LG CD4
45 late congenital syphilis 10.5 CRP CD40LG
46 breast abscess 10.4 CRP CD4
47 congenital syphilis 10.4 CD40LG CD4
48 acalculous cholecystitis 10.4 CRP CD40LG
49 multicentric reticulohistiocytosis 10.4 SERPINA3 CD1E
50 pyomyositis 10.4 CRP CD4

Comorbidity relations with Polyclonal Hypergammaglobulinemia via Phenotypic Disease Network (PDN):


Acute Cystitis Deficiency Anemia
Heart Disease

Graphical network of the top 20 diseases related to Polyclonal Hypergammaglobulinemia:



Diseases related to Polyclonal Hypergammaglobulinemia

Symptoms & Phenotypes for Polyclonal Hypergammaglobulinemia

MGI Mouse Phenotypes related to Polyclonal Hypergammaglobulinemia:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.32 CD4 CD40LG CD8A CRP IL6 MME

Drugs & Therapeutics for Polyclonal Hypergammaglobulinemia

Drugs for Polyclonal Hypergammaglobulinemia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 7)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunologic Factors
2 Rho(D) Immune Globulin
3 Immunoglobulins, Intravenous
4 gamma-Globulins
5 Antibodies
6 Immunoglobulins
7 Autoantibodies

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Gammaglobulins Level is Predictive Factor of Evolution in Sarcoidosis Completed NCT03259282
2 Prevalence of Autoantibodies in the Gaucher Disease and the Role of CD1 Molecules in Immune Manifestations of This Disease Completed NCT02650219

Search NIH Clinical Center for Polyclonal Hypergammaglobulinemia

Genetic Tests for Polyclonal Hypergammaglobulinemia

Anatomical Context for Polyclonal Hypergammaglobulinemia

MalaCards organs/tissues related to Polyclonal Hypergammaglobulinemia:

40
T Cells, Bone, B Cells, Lung, Skin, Lymph Node, Bone Marrow

Publications for Polyclonal Hypergammaglobulinemia

Articles related to Polyclonal Hypergammaglobulinemia:

(show top 50) (show all 347)
# Title Authors PMID Year
1
Idiopathic plasmacytic lymphadenopathy with polyclonal hypergammaglobulinemia accompanied with cutaneous involvement and renal dysfunction. 54 61
19586961 2009
2
High ratio of IgG4-positive plasma cell infiltration in cutaneous plasmacytosis--is this a cutaneous manifestation of IgG4-related disease? 54 61
19386351 2009
3
Cutaneous plasmacytosis with mast cell infiltration. 61
30073990 2020
4
Mutual inhibition between Prkd2 and Bcl6 controls T follicular helper cell differentiation. 61
31980486 2020
5
Primary cutaneous plasmacytosis: a case report. 61
31855272 2019
6
[Current standard in diagnostic and therapy of peripheral T-cell lymphoma]. 61
31594013 2019
7
Conditions associated with polyclonal hypergammaglobulinemia in the IgG4-related disease era: a retrospective study from a hematology tertiary care center. 61
31248968 2019
8
IgG4-related disease: what a hematologist needs to know. 61
30705099 2019
9
Immunoglobulin abnormalities are frequent in patients with lupus nephritis. 61
31453435 2019
10
[Hyper-IL-6 syndrome mimicking IgG4-related disease]. 61
31168001 2019
11
Hellenic Association for the Study of the Liver Clinical Practice Guidelines: Autoimmune hepatitis. 61
30598587 2019
12
A new presentation of isolated cutaneous Rosai-Dorfman disease: Eruptive xanthoma-like lesions. 61
30333358 2018
13
ADA2 Deficiency Mimicking Idiopathic Multicentric Castleman Disease. 61
30139808 2018
14
Myalgia caused by chronic myositis associated with plasmacytosis: a case report. 61
30107829 2018
15
[Evans syndrome complicated with multicentric Castleman disease successfully treated with tocilizumab]. 61
30185717 2018
16
Utility of Serum IgG4 Levels in a Multiethnic Population. 61
29289265 2018
17
A possible new morphological variant of mantle cell lymphoma with plasma-cell type Castleman disease-like features. 61
28974340 2017
18
Pulmonary MALT Lymphoma in Patients with Sjögren's Syndrome. 61
28487450 2017
19
[Acid sphingomyelinase deficiency (Niemann-Pick disease type B) in adulthood: A retrospective multicentric study of 28 adult cases]. 61
27884455 2017
20
Solitary ulcerated plaque on the face - an unusual presentation of cutaneous plasmacytosis? 61
29186262 2017
21
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. 61
28087540 2017
22
Systemic lupus erythematosus as the concomitant manifestation of angioimmunoblastic T-cell lymphoma. 61
25401231 2017
23
Angioimmunoblastic T-Cell Lymphoma Mimicking Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS Syndrome). 61
28611626 2017
24
Overlap of IgG4-related Disease and Multicentric Castleman's Disease in a Patient with Skin Lesions. 61
28458319 2017
25
Multicentric Castleman disease mimicking IgG4-related disease: A case report. 61
25528859 2017
26
Mucosal IgA Responses: Damaged in Established HIV Infection-Yet, Effective Weapon against HIV Transmission. 61
29176985 2017
27
IL-5 in the plasma-cell-dominant Castleman disease: a nosological entity. 61
29497544 2016
28
A Case of Lethal Idiopathic Plasmacytic Lymphadenopathy with Polyclonal Hypergammaglobulinemia: A Medical Challenge for the Forensic Pathologist. 61
27364300 2016
29
Primary Cutaneous Plasmacytosis: Masquerading as Hidradenitis Suppurativa. 61
27057027 2016
30
[Autoimmune hepatitis triggered by nitrofurantoin: A rare drug-induced toxicity]. 61
26404522 2016
31
Mercaptopurine Treatment in an Adult Man with Orbital and Intracranial Rosai-Dorfman Disease. 61
27840753 2016
32
IgG4 Related Syndrome: Another Multiorgan Disease in the Interest Field of Internal Medicine. 61
27790959 2016
33
Cutaneous and Systemic Plasmacytosis Associated with Renal Amyloidosis. 61
26719649 2015
34
Lack of cross-protection against Mycoplasma haemofelis infection and signs of enhancement in "Candidatus Mycoplasma turicensis"-recovered cats. 61
26403079 2015
35
Altered Distribution of Peripheral Blood Maturation-Associated B-Cell Subsets in Chronic Alcoholism. 61
26146763 2015
36
Inflammatory Pseudotumor of the Spleen. 61
26331003 2015
37
Angioimmunoblastic T-Cell Lymphoma Presenting with an Acute Serologic Epstein-Barr Virus Profile. 61
26331002 2015
38
Co-Existence of Multicentric Castleman's Disease and Kaposi's Sarcoma. 61
26120185 2015
39
Systemic sarcoidosis complicated of acute renal failure: about 12 cases. 61
26834928 2015
40
Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge. 61
25705537 2015
41
A case of HIV-associated diffuse infiltrative lymphocytosis syndrome simulating primary Sjögren syndrome and BALT Lymphoma. 61
25340658 2015
42
[Angioimmunoblastic T-cell lymphoma]. 61
24724413 2014
43
Epidemiology of primary Sjögren's syndrome in a French multiracial/multiethnic area. 61
23983119 2014
44
Hypertrophic pachymeningitis associated with cerebral spinal fluid hypovolemia as initial presentation of systemic lupus erythematous. 61
24351279 2014
45
IgG4-Related Disease: A Multispecialty Condition. 61
25506457 2014
46
Cutaneous and systemic plasmocytosis. 61
23937389 2013
47
Case report of intracranial Rosai-Dorfman disease. 61
24145594 2013
48
Pulmonary hypertension secondary to hyperviscosity in a patient with rheumatoid arthritis and acquired von Willebrand disease: a case report. 61
24088343 2013
49
Angioimmunoblastic T-cell lymphoma-associated pure red cell aplasia with abdominal pain. 61
23936760 2013
50
[Multicentric hyaline vascular Castleman's disease. A POEMS type variant]. 61
24021080 2013

Variations for Polyclonal Hypergammaglobulinemia

Expression for Polyclonal Hypergammaglobulinemia

Search GEO for disease gene expression data for Polyclonal Hypergammaglobulinemia.

Pathways for Polyclonal Hypergammaglobulinemia

GO Terms for Polyclonal Hypergammaglobulinemia

Cellular components related to Polyclonal Hypergammaglobulinemia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 integral component of plasma membrane GO:0005887 9.7 MME CD8A CD40LG CD4 CD1E CD1C
2 endosome GO:0005768 9.65 SLC29A3 CD1E CD1C CD1B CD1A
3 extracellular space GO:0005615 9.61 TNFSF13B SERPINA3 IL6 CRP CD40LG CD1E
4 external side of plasma membrane GO:0009897 9.17 CD8A CD40LG CD4 CD1E CD1C CD1B

Biological processes related to Polyclonal Hypergammaglobulinemia according to GeneCards Suite gene sharing:

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.95 TNFSF13B CD8A CD4 CD1E CD1C CD1B
2 adaptive immune response GO:0002250 9.85 CD4 CD1E CD1C CD1B CD1A
3 regulation of immune response GO:0050776 9.8 TNFSF13B CD8A CD40LG CD1C CD1B CD1A
4 positive regulation of T cell proliferation GO:0042102 9.67 TNFSF13B IL6 CD40LG CD4
5 acute-phase response GO:0006953 9.61 SERPINA3 IL6 CRP
6 immune response GO:0006955 9.61 TNFSF13B IL6 CD8A CD40LG CD4 CD1E
7 positive regulation of T cell mediated cytotoxicity GO:0001916 9.56 CD1E CD1C CD1B CD1A
8 positive regulation of viral entry into host cell GO:0046598 9.51 TRIM21 CD4
9 negative regulation of lipid storage GO:0010888 9.49 IL6 CRP
10 immunoglobulin secretion GO:0048305 9.48 TNFSF13B CD40LG
11 antigen processing and presentation, exogenous lipid antigen via MHC class Ib GO:0048007 9.46 CD1E CD1C CD1B CD1A
12 antigen processing and presentation, endogenous lipid antigen via MHC class Ib GO:0048006 8.92 CD1E CD1C CD1B CD1A

Molecular functions related to Polyclonal Hypergammaglobulinemia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 lipopeptide binding GO:0071723 9.46 CD1E CD1C CD1B CD1A
2 exogenous lipid antigen binding GO:0030884 9.26 CD1E CD1C CD1B CD1A
3 endogenous lipid antigen binding GO:0030883 8.92 CD1E CD1C CD1B CD1A

Sources for Polyclonal Hypergammaglobulinemia

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
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