MCID: GDS001
MIFTS: 41

Good Syndrome

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Good Syndrome

MalaCards integrated aliases for Good Syndrome:

Name: Good Syndrome 12 60 15
Thymoma with Hypogammaglobulinemia 12
Thymoma-Immunodeficiency Syndrome 60
Immunodeficiency with Thymoma 74

Characteristics:

Orphanet epidemiological data:

60
good syndrome
Age of onset: Adult; Age of death: Not yet documented;

Classifications:

Orphanet: 60  
Rare immunological diseases


Summaries for Good Syndrome

Disease Ontology : 12 An agammaglobulinemia that is a condition where there is co-occurrence of deficient cell-mediated immunity and benign thymoma.

MalaCards based summary : Good Syndrome, also known as thymoma with hypogammaglobulinemia, is related to immunodeficiency with thymoma and chronic granulomatous disease. An important gene associated with Good Syndrome is EPHB6 (EPH Receptor B6), and among its related pathways/superpathways are T Cell Co-Signaling Pathway: Ligand-Receptor Interactions and B Cell Development Pathways. Affiliated tissues include skin, thymus and bone, and related phenotypes are decreased antibody level in blood and thymoma

Related Diseases for Good Syndrome

Graphical network of the top 20 diseases related to Good Syndrome:



Diseases related to Good Syndrome

Symptoms & Phenotypes for Good Syndrome

Human phenotypes related to Good Syndrome:

60 33 (show all 21)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 decreased antibody level in blood 60 33 hallmark (90%) Very frequent (99-80%) HP:0004313
2 thymoma 60 33 hallmark (90%) Very frequent (99-80%) HP:0100522
3 mediastinal lymphadenopathy 60 33 hallmark (90%) Very frequent (99-80%) HP:0100721
4 ptosis 60 33 frequent (33%) Frequent (79-30%) HP:0000508
5 dysphonia 60 33 frequent (33%) Frequent (79-30%) HP:0001618
6 dysphagia 60 33 frequent (33%) Frequent (79-30%) HP:0002015
7 dyspnea 60 33 frequent (33%) Frequent (79-30%) HP:0002094
8 sinusitis 60 33 frequent (33%) Frequent (79-30%) HP:0000246
9 cough 60 33 frequent (33%) Frequent (79-30%) HP:0012735
10 recurrent urinary tract infections 60 33 frequent (33%) Frequent (79-30%) HP:0000010
11 recurrent skin infections 60 33 frequent (33%) Frequent (79-30%) HP:0001581
12 bronchiectasis 60 33 frequent (33%) Frequent (79-30%) HP:0002110
13 fatigable weakness 60 33 frequent (33%) Frequent (79-30%) HP:0003473
14 abnormal leukocyte morphology 33 frequent (33%) HP:0001881
15 diabetes mellitus 60 33 occasional (7.5%) Occasional (29-5%) HP:0000819
16 recurrent respiratory infections 60 33 occasional (7.5%) Occasional (29-5%) HP:0002205
17 anemia 60 33 occasional (7.5%) Occasional (29-5%) HP:0001903
18 thrombocytopenia 60 33 occasional (7.5%) Occasional (29-5%) HP:0001873
19 aplasia/hypoplasia of the thymus 60 33 occasional (7.5%) Occasional (29-5%) HP:0010515
20 diarrhea 60 33 occasional (7.5%) Occasional (29-5%) HP:0002014
21 abnormality of leukocytes 60 Frequent (79-30%)

MGI Mouse Phenotypes related to Good Syndrome:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.63 CD40LG DTX1 EPHB6 ICOS STX11 UNC13D
2 homeostasis/metabolism MP:0005376 9.5 CD40LG DTX1 EPHA1 ICOS SEL1L STX11
3 immune system MP:0005387 9.17 CD40LG DTX1 EPHA1 EPHB6 ICOS STX11

Drugs & Therapeutics for Good Syndrome

Search Clinical Trials , NIH Clinical Center for Good Syndrome

Genetic Tests for Good Syndrome

Anatomical Context for Good Syndrome

MalaCards organs/tissues related to Good Syndrome:

42
Skin, Thymus, Bone, Bone Marrow, T Cells, B Cells

Publications for Good Syndrome

Articles related to Good Syndrome:

(show top 50) (show all 54)
# Title Authors Year
1
Precursor B-cell development in bone marrow of Good syndrome patients. ( 30690192 )
2019
2
Prevention of infectious diseases in patients with Good syndrome. ( 29878906 )
2018
3
Severe diarrhoea due to Cystoisospora belli infection in a Good syndrome patient. ( 29625163 )
2018
4
Features and outcomes of immunoglobulin therapy in patients with Good syndrome at Thailand's largest tertiary referral hospital. ( 29888926 )
2018
5
Aberrant Peripheral Immune Function in a Good Syndrome Patient. ( 29850635 )
2018
6
Good syndrome: immunodeficiency associated with thymoma. ( 29984501 )
2018
7
Agranulocytosis and Good Syndrome in Patient with Thymoma-The Role of Immunosuppressive Treatment after Thymectomy. ( 30166018 )
2018
8
Thymoma-associated multi-organ autoimmunity: A case of graft-versus-host disease-like erythroderma complicated by Good syndrome successfully treated by thymectomy. ( 28256733 )
2017
9
Thymoma with immunodeficiency/Good syndrome associated with myasthenia gravis. ( 28450687 )
2017
10
Pure Red Cell Aplasia Associated with Good Syndrome. ( 28382272 )
2017
11
Cytomegalovirus Retinitis as the First Manifestation of Good Syndrome. ( 27715373 )
2016
12
Good syndrome and other causes of cytomegalovirus retinitis in HIV-negative patients-case report and comprehensive review of the literature. ( 26809342 )
2016
13
Atypical VZV Retinitis in a Patient with Good Syndrome. ( 27494574 )
2016
14
Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective. ( 27497628 )
2016
15
Tuberculous lymphadenitis in a patient with Good syndrome. ( 27487062 )
2016
16
Three difficult cases: the challenge of autoimmunity, immunodeficiency and recurrent infections in patients with Good syndrome. ( 25059810 )
2015
17
Cytokine profiles contribute to understanding the pathogenic difference between good syndrome and oral lichen planus: two case reports and literature review. ( 25860215 )
2015
18
Good Syndrome: An Adult-Onset Immunodeficiency Remarkable for Its High Incidence of Invasive Infections and Autoimmune Complications. ( 25828999 )
2015
19
Good syndrome presenting with CD8+ T-Cell large granular lymphocyte leukemia. ( 26429871 )
2015
20
A case of good syndrome presumed secondary to metastatic pancreatic thymoma in a patient presenting with a myasthenic crisis postthymectomy. ( 25695921 )
2015
21
Good syndrome and polymyositis. ( 24685393 )
2014
22
Good syndrome and lichen planus: case and review. ( 24588858 )
2014
23
Good syndrome, bad problem. ( 25426445 )
2014
24
Treatment of mucosal leishmaniasis (L. infantum) with miltefosine in a patient with Good syndrome. ( 23871798 )
2013
25
Thymoma and immunodeficiency: (18)F-FDG-PET/CT imaging of Good syndrome. ( 23687646 )
2013
26
Autoimmune retinopathy in benign thymoma after good syndrome-associated cytomegalovirus retinitis. ( 23323587 )
2013
27
Good Syndrome, a rare cause of refractory chronic diarrhea and recurrent pneumonia in a Chinese patient after thymectomy. ( 23697574 )
2013
28
TACI mutation p.Lys154Ter identified in Good Syndrome. ( 23165285 )
2013
29
Good syndrome, a rare disease that physicians cannot afford to overlook; case report and review of literature. ( 24397104 )
2013
30
A case of Good syndrome with pulmonary lesions similar to diffuse panbronchiolitis. ( 22576393 )
2012
31
A case of Good syndrome with refractory gastrointestinal ulcers. ( 22715016 )
2012
32
Bilateral cytomegalovirus retinitis with unilateral optic neuritis in Good syndrome. ( 20577863 )
2010
33
A rare combination of recurrent pneumonia, diarrhoea, and visual loss in a patient after thymectomy: good syndrome. ( 21135430 )
2010
34
Interleukin-2-unresponsive immune defects in good syndrome: Letter to the Editor. ( 20185370 )
2010
35
Malignant thymoma with immunodeficiency (Good syndrome) associated with mucormycosis. ( 20139744 )
2010
36
Cytomegalovirus retinitis in Good syndrome: case report and review of literature. ( 21691402 )
2009
37
Durable hypogammaglobulinemia associated with thymoma (Good syndrome). ( 19797831 )
2009
38
Sclerosing cholangitis associated with good syndrome. ( 20128430 )
2009
39
Good syndrome accompanied by pure red cell aplasia. ( 19608562 )
2009
40
Type AB thymoma accompanied by pure red cell aplasia and Good syndrome with CMV infection of tumor cells. ( 18705768 )
2008
41
Myelodysplasia and Good syndrome. A case report. ( 18791691 )
2008
42
Thymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of the literature. ( 17304889 )
2007
43
Good syndrome coexisting with leukopenia. ( 18036947 )
2007
44
Good syndrome with thymic adenosquamous carcinoma--report of a case. ( 17392673 )
2007
45
Periodic myoclonus due to cytomegalovirus encephalitis in a patient with good syndrome. ( 17296846 )
2007
46
A case of oral lichen planus with Good syndrome. ( 17083908 )
2006
47
CMV retinitis in a patient with good syndrome. ( 16321895 )
2005
48
Successful treatment of Good syndrome with cytomegalovirus duodenoenteritis using a combination of ganciclovir and immunoglobulin with high anti-cytomegalovirus antibody titer. ( 14722398 )
2004
49
Fatal pneumocystis pneumonia with good syndrome and pure red cell aplasia. ( 15578389 )
2004
50
[Hypogammaglobulinemia associated with thymoma (Good syndrome) similar to diffuse panbronchiolitis]. ( 12833850 )
2003

Variations for Good Syndrome

Expression for Good Syndrome

Search GEO for disease gene expression data for Good Syndrome.

Pathways for Good Syndrome

Pathways related to Good Syndrome according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.92 CD40LG ICOS
2 10.68 CD40LG ICOS
3 10.34 CD40LG EPHA1 EPHB6
4 10.19 CD40LG ICOS

GO Terms for Good Syndrome

Cellular components related to Good Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 integral component of plasma membrane GO:0005887 8.92 CD40LG EPHA1 EPHB6 ICOS

Biological processes related to Good Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.16 EPHA1 EPHB6
2 ephrin receptor signaling pathway GO:0048013 8.96 EPHA1 EPHB6
3 T cell costimulation GO:0031295 8.62 CD40LG ICOS

Molecular functions related to Good Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.16 EPHA1 EPHB6
2 transmembrane-ephrin receptor activity GO:0005005 8.96 EPHA1 EPHB6
3 ephrin receptor activity GO:0005003 8.62 EPHA1 EPHB6

Sources for Good Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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