MCID: GDS001
MIFTS: 40

Good Syndrome

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Good Syndrome

MalaCards integrated aliases for Good Syndrome:

Name: Good Syndrome 12 59 15
Thymoma with Hypogammaglobulinemia 12
Thymoma-Immunodeficiency Syndrome 59
Immunodeficiency with Thymoma 72

Characteristics:

Orphanet epidemiological data:

59
good syndrome
Age of onset: Adult; Age of death: Not yet documented;

Classifications:

Orphanet: 59  
Rare immunological diseases


External Ids:

Disease Ontology 12 DOID:0060028
UMLS via Orphanet 73 C0221027
Orphanet 59 ORPHA169105
UMLS 72 C0221027

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 receptor signaling pathway and IL12-mediated signaling events. Affiliated tissues include skin, thymus and bone, and related phenotypes are decreased antibody level in blood and thymoma

Wikipedia : 75 Thymoma with immunodeficiency (also known as "Good syndrome") is a rare disorder that occurs in adults... more...

Related Diseases for Good Syndrome

Diseases related to Good Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 98)
# Related Disease Score Top Affiliating Genes
1 immunodeficiency with thymoma 12.0
2 chronic granulomatous disease 11.7
3 thymoma, familial 10.3
4 thymoma 10.3
5 agammaglobulinemia 10.2
6 immune deficiency disease 10.0
7 neuroretinitis 10.0
8 pure red-cell aplasia 10.0
9 retinitis 10.0
10 red cell aplasia 10.0
11 cytomegalovirus retinitis 10.0
12 pyoderma 10.0
13 alopecia areata 10.0
14 alopecia 10.0
15 lichen planus 9.9
16 candidiasis 9.9
17 myasthenia gravis 9.9
18 herpes simplex 9.9
19 metal allergy 9.9 CD8A CD4
20 polyclonal hypergammaglobulinemia 9.8 CD8A CD4
21 autoimmune disease 9.8
22 cd4/cd8 t-cell ratio 9.8
23 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.8
24 diarrhea 9.8
25 lymphopenia 9.8
26 thymus lipoma 9.8 CD8A CD4
27 panbronchiolitis, diffuse 9.8
28 bacterial infectious disease 9.8
29 common variable immunodeficiency 9.8
30 neutropenia 9.8
31 thymic carcinoma 9.8
32 pneumonia 9.8
33 chickenpox 9.8
34 oral lichen planus 9.8
35 immune system organ benign neoplasm 9.7 CD8A CD4
36 kaposi sarcoma 9.7
37 combined t cell and b cell immunodeficiency 9.7
38 bacterial pneumonia 9.7
39 cytomegalovirus infection 9.7
40 xanthogranulomatous cholecystitis 9.6 CD8A CD4
41 keratitis, hereditary 9.5
42 otitis media 9.5
43 retinal detachment 9.5
44 agammaglobulinemia, x-linked 9.5
45 pars planitis 9.5
46 kala-azar 1 9.5
47 aspergillosis 9.5
48 myelodysplastic syndrome 9.5
49 graft-versus-host disease 9.5
50 human herpesvirus 8 9.5

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:

59 32 (show all 21)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 decreased antibody level in blood 59 32 hallmark (90%) Very frequent (99-80%) HP:0004313
2 thymoma 59 32 hallmark (90%) Very frequent (99-80%) HP:0100522
3 mediastinal lymphadenopathy 59 32 hallmark (90%) Very frequent (99-80%) HP:0100721
4 ptosis 59 32 frequent (33%) Frequent (79-30%) HP:0000508
5 dysphonia 59 32 frequent (33%) Frequent (79-30%) HP:0001618
6 dysphagia 59 32 frequent (33%) Frequent (79-30%) HP:0002015
7 dyspnea 59 32 frequent (33%) Frequent (79-30%) HP:0002094
8 sinusitis 59 32 frequent (33%) Frequent (79-30%) HP:0000246
9 recurrent urinary tract infections 59 32 frequent (33%) Frequent (79-30%) HP:0000010
10 cough 59 32 frequent (33%) Frequent (79-30%) HP:0012735
11 recurrent skin infections 59 32 frequent (33%) Frequent (79-30%) HP:0001581
12 bronchiectasis 59 32 frequent (33%) Frequent (79-30%) HP:0002110
13 fatigable weakness 59 32 frequent (33%) Frequent (79-30%) HP:0003473
14 abnormal leukocyte morphology 32 frequent (33%) HP:0001881
15 diabetes mellitus 59 32 occasional (7.5%) Occasional (29-5%) HP:0000819
16 recurrent respiratory infections 59 32 occasional (7.5%) Occasional (29-5%) HP:0002205
17 anemia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001903
18 thrombocytopenia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001873
19 aplasia/hypoplasia of the thymus 59 32 occasional (7.5%) Occasional (29-5%) HP:0010515
20 diarrhea 59 32 occasional (7.5%) Occasional (29-5%) HP:0002014
21 abnormality of leukocytes 59 Frequent (79-30%)

MGI Mouse Phenotypes related to Good Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.85 CD4 CD8A DTX1 EPHB6 SEL1L TCOF1
2 endocrine/exocrine gland MP:0005379 9.73 CD4 CD8A DTX1 ICOS SEL1L TCOF1
3 hematopoietic system MP:0005397 9.63 CD4 CD8A DTX1 EPHB6 ICOS STX11
4 digestive/alimentary MP:0005381 9.62 CD4 ICOS SEL1L TCOF1
5 immune system MP:0005387 9.43 CD4 CD8A DTX1 EPHB6 ICOS STX11
6 no phenotypic analysis MP:0003012 8.92 CD4 EPHB6 ICOS STX11

Drugs & Therapeutics for Good Syndrome

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 The Association of Depression With "Motoric Cognitive Risk" Syndrome in the Canadian Population Active, not recruiting NCT03679221

Search NIH Clinical Center for Good Syndrome

Genetic Tests for Good Syndrome

Anatomical Context for Good Syndrome

MalaCards organs/tissues related to Good Syndrome:

41
Skin, Thymus, Bone, Bone Marrow, T Cells, B Cells

Publications for Good Syndrome

Articles related to Good Syndrome:

(show top 50) (show all 85)
# Title Authors PMID Year
1
Features and outcomes of immunoglobulin therapy in patients with Good syndrome at Thailand's largest tertiary referral hospital. 38
29888926 2019
2
Thymic epithelial tumor complicated by immunological abnormalities: results from a single-center retrospective study in China. 38
31179102 2019
3
Thymic neoplasms patients complicated with bronchiectasis: Case series in a Chinese hospital and literature review. 38
30758142 2019
4
Precursor B-cell development in bone marrow of Good syndrome patients. 38
30690192 2019
5
Humoral immunodeficiencies: conferred risk of infections and benefits of immunoglobulin replacement therapy. 38
30536429 2018
6
Agranulocytosis and Good Syndrome in Patient with Thymoma-The Role of Immunosuppressive Treatment after Thymectomy. 38
30166018 2018
7
Prevention of infectious diseases in patients with Good syndrome. 38
29878906 2018
8
Severe diarrhoea due to Cystoisospora belli infection in a Good syndrome patient. 38
29625163 2018
9
Good syndrome: immunodeficiency associated with thymoma. 38
29984501 2018
10
Delayed Diagnosis and Complications of Predominantly Antibody Deficiencies in a Cohort of Australian Adults. 38
29867917 2018
11
Atypical VZV Retinitis in a Patient with Good Syndrome. 38
27494574 2018
12
Aberrant Peripheral Immune Function in a Good Syndrome Patient. 38
29850635 2018
13
Cytomegalovirus Retinitis as the First Manifestation of Good Syndrome. 38
27715373 2018
14
Thymoma-associated multi-organ autoimmunity: A case of graft-versus-host disease-like erythroderma complicated by Good syndrome successfully treated by thymectomy. 38
28256733 2017
15
Thymoma with immunodeficiency/Good syndrome associated with myasthenia gravis. 38
28450687 2017
16
[Good's syndrome and congenital toxoplasmosis due to maternal reactivation during pregnancy]. 38
28347510 2017
17
Ecthyma gangrenosum without bacteraemia: evidence in favour of a broader definition. 38
28239902 2017
18
Pure Red Cell Aplasia Associated with Good Syndrome. 38
28382272 2017
19
[Acquired hypogammaglobulinemia associated with thymoma: Good syndrome]. 38
29881497 2017
20
Good syndrome and other causes of cytomegalovirus retinitis in HIV-negative patients-case report and comprehensive review of the literature. 38
26809342 2016
21
Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective. 38
27497628 2016
22
Tuberculous lymphadenitis in a patient with Good syndrome. 38
27487062 2016
23
Thymic Epithelial Tumor-Associated Cytopenia: A 10-Year Observational Study in France. 38
26768832 2016
24
Good syndrome presenting with CD8⁺ T-Cell large granular lymphocyte leukemia. 38
26429871 2015
25
Good syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complications. 38
25828999 2015
26
Cytokine profiles contribute to understanding the pathogenic difference between Good syndrome and oral lichen planus: two case reports and literature review. 38
25860215 2015
27
A case of good syndrome presumed secondary to metastatic pancreatic thymoma in a patient presenting with a myasthenic crisis postthymectomy. 38
25695921 2015
28
Three difficult cases: the challenge of autoimmunity, immunodeficiency and recurrent infections in patients with Good syndrome. 38
25059810 2015
29
Good syndrome and lichen planus: case and review. 38
24588858 2014
30
Good syndrome and polymyositis. 38
24685393 2014
31
Good syndrome, bad problem. 38
25426445 2014
32
Good syndrome, a rare disease that physicians cannot afford to overlook; case report and review of literature. 38
24397104 2013
33
Treatment of mucosal leishmaniasis (L. infantum) with miltefosine in a patient with Good syndrome. 38
23871798 2013
34
Good Syndrome, a rare cause of refractory chronic diarrhea and recurrent pneumonia in a Chinese patient after thymectomy. 38
23697574 2013
35
Thymoma and immunodeficiency: (18)F-FDG-PET/CT imaging of Good syndrome. 38
23687646 2013
36
Autoimmune retinopathy in benign thymoma after good syndrome-associated cytomegalovirus retinitis. 38
23323587 2013
37
TACI mutation p.Lys154Ter identified in Good Syndrome. 38
23165285 2013
38
[Association of Good syndrome with pure red cell aplasia: report of one case]. 38
23282780 2012
39
A case of Good syndrome with pulmonary lesions similar to diffuse panbronchiolitis. 38
22576393 2012
40
A case of Good syndrome with refractory gastrointestinal ulcers. 38
22715016 2012
41
[A Good syndrome associated with pure red cell aplasia]. 38
22073609 2011
42
[Glossitis by mixed infection of cytomegalovirus and herpes simplex virus during therapy for pure red cell aplasia complicated with Good syndrome]. 38
21897079 2011
43
Diagnostic strategy for patients with hypogammaglobulinemia in rheumatology. 38
21036646 2011
44
[Antibody deficiencies. A survey from three Clinical Immunology Centers for adults in Buenos Aires city]. 38
21893448 2011
45
A rare combination of recurrent pneumonia, diarrhoea, and visual loss in a patient after thymectomy: good syndrome. 38
21135430 2010
46
[Oral erosive lichen planus associated with Good syndrome]. 38
20494493 2010
47
Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence. 38
20149753 2010
48
Interleukin-2-unresponsive immune defects in good syndrome: Letter to the Editor. 38
20185370 2010
49
Bilateral cytomegalovirus retinitis with unilateral optic neuritis in Good syndrome. 38
20577863 2010
50
Malignant thymoma with immunodeficiency (Good syndrome) associated with mucormycosis. 38
20139744 2010

Variations for Good Syndrome

Expression for Good Syndrome

Search GEO for disease gene expression data for Good Syndrome.

Pathways for Good Syndrome

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 9.46 ICOS EPHB6 CD8A CD4
2 external side of plasma membrane GO:0009897 9.13 ICOS CD8A CD4
3 T cell receptor complex GO:0042101 8.62 CD8A CD4

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.5 ICOS CD8A CD4
2 cell surface receptor signaling pathway GO:0007166 9.33 DTX1 CD8A CD4
3 T cell activation GO:0042110 9.26 CD8A CD4
4 T cell mediated immunity GO:0002456 8.96 EPHB6 CD8A
5 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 8.8 EPHB6 CD8A CD4

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

# Name GO ID Score Top Affiliating Genes
1 coreceptor activity GO:0015026 8.62 CD8A CD4

Sources for Good Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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