MCID: GDS001
MIFTS: 38

Good Syndrome

Categories: Blood diseases, Rare diseases, Immune 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 73

Characteristics:

Orphanet epidemiological data:

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

Classifications:

Orphanet: 59  
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). Affiliated tissues include thymus, skin and t cells, and related phenotypes are recurrent urinary tract infections and sinusitis

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:

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

MGI Mouse Phenotypes related to Good Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 9.63 EPHA1 EPHB6 LRP4 MUC5B SEL1L STX11
2 homeostasis/metabolism MP:0005376 9.5 EPHA1 ICOS LRP4 MUC5B SEL1L STX11
3 immune system MP:0005387 9.1 EPHA1 EPHB6 ICOS MUC5B STX11 UNC13D

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:

41
Thymus, Skin, T Cells

Publications for Good Syndrome

Articles related to Good Syndrome:

(show top 50) (show all 51)
# Title Authors Year
1
Prevention of infectious diseases in patients with Good syndrome. ( 29878906 )
2018
2
Severe diarrhoea due to Cystoisospora belli infection in a Good syndrome patient. ( 29625163 )
2018
3
Features and outcomes of immunoglobulin therapy in patients with Good syndrome at Thailand's largest tertiary referral hospital. ( 29888926 )
2018
4
Aberrant Peripheral Immune Function in a Good Syndrome Patient. ( 29850635 )
2018
5
Thymoma-associated multi-organ autoimmunity: A case of graft-versus-host disease-like erythroderma complicated by Good syndrome successfully treated by thymectomy. ( 28256733 )
2017
6
Thymoma with immunodeficiency/Good syndrome associated with myasthenia gravis. ( 28450687 )
2017
7
Pure Red Cell Aplasia Associated with Good Syndrome. ( 28382272 )
2017
8
Cytomegalovirus Retinitis as the First Manifestation of Good Syndrome. ( 27715373 )
2016
9
Good syndrome and other causes of cytomegalovirus retinitis in HIV-negative patients-case report and comprehensive review of the literature. ( 26809342 )
2016
10
Atypical VZV Retinitis in a Patient with Good Syndrome. ( 27494574 )
2016
11
Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective. ( 27497628 )
2016
12
Tuberculous lymphadenitis in a patient with Good syndrome. ( 27487062 )
2016
13
Three difficult cases: the challenge of autoimmunity, immunodeficiency and recurrent infections in patients with Good syndrome. ( 25059810 )
2015
14
Cytokine profiles contribute to understanding the pathogenic difference between good syndrome and oral lichen planus: two case reports and literature review. ( 25860215 )
2015
15
Good Syndrome: An Adult-Onset Immunodeficiency Remarkable for Its High Incidence of Invasive Infections and Autoimmune Complications. ( 25828999 )
2015
16
Good syndrome presenting with CD8+ T-Cell large granular lymphocyte leukemia. ( 26429871 )
2015
17
A case of good syndrome presumed secondary to metastatic pancreatic thymoma in a patient presenting with a myasthenic crisis postthymectomy. ( 25695921 )
2015
18
Good syndrome and polymyositis. ( 24685393 )
2014
19
Good syndrome and lichen planus: case and review. ( 24588858 )
2014
20
Good syndrome, bad problem. ( 25426445 )
2014
21
Treatment of mucosal leishmaniasis (L. infantum) with miltefosine in a patient with Good syndrome. ( 23871798 )
2013
22
Thymoma and immunodeficiency: (18)F-FDG-PET/CT imaging of Good syndrome. ( 23687646 )
2013
23
Autoimmune retinopathy in benign thymoma after good syndrome-associated cytomegalovirus retinitis. ( 23323587 )
2013
24
Good Syndrome, a rare cause of refractory chronic diarrhea and recurrent pneumonia in a Chinese patient after thymectomy. ( 23697574 )
2013
25
TACI mutation p.Lys154Ter identified in Good Syndrome. ( 23165285 )
2013
26
Good syndrome, a rare disease that physicians cannot afford to overlook; case report and review of literature. ( 24397104 )
2013
27
A case of Good syndrome with pulmonary lesions similar to diffuse panbronchiolitis. ( 22576393 )
2012
28
A case of Good syndrome with refractory gastrointestinal ulcers. ( 22715016 )
2012
29
Bilateral cytomegalovirus retinitis with unilateral optic neuritis in Good syndrome. ( 20577863 )
2010
30
A rare combination of recurrent pneumonia, diarrhoea, and visual loss in a patient after thymectomy: good syndrome. ( 21135430 )
2010
31
Interleukin-2-unresponsive immune defects in good syndrome: Letter to the Editor. ( 20185370 )
2010
32
Malignant thymoma with immunodeficiency (Good syndrome) associated with mucormycosis. ( 20139744 )
2010
33
Cytomegalovirus retinitis in Good syndrome: case report and review of literature. ( 21691402 )
2009
34
Durable hypogammaglobulinemia associated with thymoma (Good syndrome). ( 19797831 )
2009
35
Sclerosing cholangitis associated with good syndrome. ( 20128430 )
2009
36
Good syndrome accompanied by pure red cell aplasia. ( 19608562 )
2009
37
Type AB thymoma accompanied by pure red cell aplasia and Good syndrome with CMV infection of tumor cells. ( 18705768 )
2008
38
Myelodysplasia and Good syndrome. A case report. ( 18791691 )
2008
39
Thymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of the literature. ( 17304889 )
2007
40
Good syndrome coexisting with leukopenia. ( 18036947 )
2007
41
Good syndrome with thymic adenosquamous carcinoma--report of a case. ( 17392673 )
2007
42
Periodic myoclonus due to cytomegalovirus encephalitis in a patient with good syndrome. ( 17296846 )
2007
43
A case of oral lichen planus with Good syndrome. ( 17083908 )
2006
44
CMV retinitis in a patient with good syndrome. ( 16321895 )
2005
45
Successful treatment of Good syndrome with cytomegalovirus duodenoenteritis using a combination of ganciclovir and immunoglobulin with high anti-cytomegalovirus antibody titer. ( 14722398 )
2004
46
Fatal pneumocystis pneumonia with good syndrome and pure red cell aplasia. ( 15578389 )
2004
47
[Hypogammaglobulinemia associated with thymoma (Good syndrome) similar to diffuse panbronchiolitis]. ( 12833850 )
2003
48
Recurrent pneumonia post-thymectomy as a manifestation of Good syndrome. ( 12554014 )
2003
49
Clinicopathologic reports, case reports, and small case series: cytomegalovirus retinitis in patients with Good syndrome. ( 11934330 )
2002
50
Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. ( 11307588 )
2001

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 receptor complex GO:0043235 8.8 EPHA1 EPHB6 LRP4

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

# Name GO ID Score Top Affiliating Genes
1 ephrin receptor signaling pathway GO:0048013 8.96 EPHA1 EPHB6
2 positive regulation of kinase activity GO:0033674 8.62 EPHA1 EPHB6

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

# Name GO ID Score Top Affiliating Genes
1 MAP kinase kinase kinase activity GO:0004709 9.32 EPHA1 EPHB6
2 mitogen-activated protein kinase kinase binding GO:0031434 9.26 EPHA1 EPHB6
3 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.16 EPHA1 EPHB6
4 transmembrane-ephrin receptor activity GO:0005005 8.96 EPHA1 EPHB6
5 ephrin receptor activity GO:0005003 8.62 EPHA1 EPHB6

Sources for Good Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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