MCID: GDS001
MIFTS: 42

Good Syndrome

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Good Syndrome

MalaCards integrated aliases for Good Syndrome:

Name: Good Syndrome 12 52 58 15
Thymoma-Immunodeficiency Syndrome 52 58
Immunodeficiency with Thymoma 52 71
Thymoma with Hypogammaglobulinemia 12

Characteristics:

Orphanet epidemiological data:

58
good syndrome
Age of onset: Adult; Age of death: Not yet documented;

Classifications:

Orphanet: 58  
Rare immunological diseases


Summaries for Good Syndrome

NIH Rare Diseases : 52 Good syndrome is a rare, adult-onset primary immunodeficiency suspected in patients who exhibit hypogammaglobulinemia and low levels of B cells along with a benign thymic tumor (thymoma) on chest X-ray . Symptoms include frequent opportunistic infections involving the sinuses and lungs, including severe CMV disease , P. carinii pneumonia , and mucocutaneous candidiasis . While the cause of Good syndrome remains unknown, there is some evidence that a defect of the bone marrow is involved. Treatment includes removal of the thymic tumor and immunoglobulin replacement .

MalaCards based summary : Good Syndrome, also known as thymoma-immunodeficiency syndrome, is related to neuroretinitis and cytomegalovirus retinitis. An important gene associated with Good Syndrome is CLPSL2 (Colipase Like 2), and among its related pathways/superpathways are Akt Signaling and Allograft rejection. Affiliated tissues include lung, bone and b cells, and related phenotypes are decreased antibody level in blood and thymoma

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

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 447)
# Related Disease Score Top Affiliating Genes
1 neuroretinitis 30.4 CRP CD40LG CD4
2 cytomegalovirus retinitis 29.9 TNF CD8A CD4
3 immunodeficiency 18 29.8 CD8A CD4
4 t cell deficiency 29.8 CD8A CD40LG CD4
5 agammaglobulinemia 29.7 TNF CD40LG CD19
6 pyoderma 29.6 TNFRSF1B TNF CRP
7 combined t cell and b cell immunodeficiency 29.4 CD8A CD40LG CD4 CD19
8 keratitis, hereditary 29.1 TNF CD8A CD4
9 acquired immunodeficiency syndrome 28.9 TNFRSF1B TNF CRP CD40LG CD4
10 cholangitis 28.9 TNF CRP CD40LG
11 immune deficiency disease 28.9 TNFRSF13B TNF CD40LG CD4
12 lymphadenitis 28.9 TNF CRP CD4
13 bronchiolitis 28.8 TNF CRP CD40LG
14 bacterial infectious disease 28.7 TNF CRP CD8A CD40LG CD4
15 pneumonia 28.7 TNFRSF1B TNF CRP CD8A CD40LG
16 otitis media 28.7 TNF CRP CD40LG
17 vasculitis 28.7 TNFRSF1B TNF CRP CD40LG
18 chorioretinitis 28.6 TNFRSF1B TNF CD8A CD40LG CD4
19 bacterial pneumonia 28.6 TNF CRP CD8A CD40LG CD4
20 bronchitis 28.6 TNF CRP CD40LG
21 autoimmune disease 28.4 TNFRSF1B TNFRSF13B TNF CRP CD40LG
22 common variable immunodeficiency 28.2 TNFRSF1B TNFRSF13B TNF CD40LG CD19
23 pneumocystosis 28.1 TNFRSF1B TNF CRP CD8A CD40LG CD4
24 leukemia, chronic lymphocytic 28.0 TNFRSF13B TNF CD8A CD40LG CD4 CD19
25 chickenpox 27.9 TNFRSF1B TNF CRP CD8A CD40LG CD4
26 myeloma, multiple 27.6 TNFRSF13B TNF CRP CD8A CD40LG CD4
27 chronic granulomatous disease 11.7
28 riedel's fibrosing thyroiditis 10.5 CD40LG CD4
29 glossopharyngeal nerve paralysis 10.5 TNFRSF1B CD40LG
30 ventilation pneumonitis 10.5 CD8A CD4
31 diffuse infiltrative lymphocytosis syndrome 10.5 CD8A CD4
32 autoimmune lymphoproliferative syndrome, type iia 10.5 CD8A CD4
33 chromosome 14q11-q22 deletion syndrome 10.5 CD8A CD4
34 latent syphilis 10.5 CD40LG CD4
35 gastroduodenitis 10.5 CD40LG CD4
36 bird fancier's lung 10.5 CD8A CD4
37 primary syphilis 10.5 CD40LG CD4
38 asymptomatic neurosyphilis 10.5 CD40LG CD4
39 erythema elevatum diutinum 10.5 TNFRSF1B CD40LG
40 tertiary neurosyphilis 10.5 CD40LG CD4
41 transient arthritis 10.5 CD40LG CD4
42 meningovascular neurosyphilis 10.5 CD40LG CD4
43 parapsoriasis 10.5 CD8A CD4
44 congenital syphilis 10.4 CD40LG CD4
45 metal allergy 10.4 CD8A CD4
46 variola major 10.4 CD8A CD4
47 viral exanthem 10.4 CD40LG CD4
48 follicular mucinosis 10.4 CD8A CD4
49 spongiotic dermatitis 10.4 CD8A CD4
50 nickel allergic contact dermatitis 10.4 CD8A CD4

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:

58 31 (show all 21)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 decreased antibody level in blood 58 31 hallmark (90%) Very frequent (99-80%) HP:0004313
2 thymoma 58 31 hallmark (90%) Very frequent (99-80%) HP:0100522
3 mediastinal lymphadenopathy 58 31 hallmark (90%) Very frequent (99-80%) HP:0100721
4 ptosis 58 31 frequent (33%) Frequent (79-30%) HP:0000508
5 dysphonia 58 31 frequent (33%) Frequent (79-30%) HP:0001618
6 dysphagia 58 31 frequent (33%) Frequent (79-30%) HP:0002015
7 dyspnea 58 31 frequent (33%) Frequent (79-30%) HP:0002094
8 sinusitis 58 31 frequent (33%) Frequent (79-30%) HP:0000246
9 recurrent urinary tract infections 58 31 frequent (33%) Frequent (79-30%) HP:0000010
10 cough 58 31 frequent (33%) Frequent (79-30%) HP:0012735
11 recurrent skin infections 58 31 frequent (33%) Frequent (79-30%) HP:0001581
12 bronchiectasis 58 31 frequent (33%) Frequent (79-30%) HP:0002110
13 fatigable weakness 58 31 frequent (33%) Frequent (79-30%) HP:0003473
14 abnormal leukocyte morphology 31 frequent (33%) HP:0001881
15 diabetes mellitus 58 31 occasional (7.5%) Occasional (29-5%) HP:0000819
16 recurrent respiratory infections 58 31 occasional (7.5%) Occasional (29-5%) HP:0002205
17 anemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001903
18 thrombocytopenia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001873
19 aplasia/hypoplasia of the thymus 58 31 occasional (7.5%) Occasional (29-5%) HP:0010515
20 diarrhea 58 31 occasional (7.5%) Occasional (29-5%) HP:0002014
21 abnormality of leukocytes 58 Frequent (79-30%)

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:

40
Lung, Bone, B Cells, Bone Marrow, T Cells, Thymus, Skin

Publications for Good Syndrome

Articles related to Good Syndrome:

(show top 50) (show all 90)
# Title Authors PMID Year
1
Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence. 61 52
20149753 2010
2
A systematic review of paraneoplastic syndromes associated with thymoma: Treatment modalities, recurrence, and outcomes in resected cases. 61
31982129 2019
3
Delayed Diagnosis of Good Syndrome. 61
31839563 2019
4
Thymoma complicated with myasthenia gravis and Good syndrome - a therapeutic conundrum: a case report. 61
31779680 2019
5
A Patient With Good Syndrome Complicated With Phlegmonous Gastritis. 61
31832472 2019
6
Features and outcomes of immunoglobulin therapy in patients with Good syndrome at Thailand's largest tertiary referral hospital. 61
29888926 2019
7
Thymic epithelial tumor complicated by immunological abnormalities: results from a single-center retrospective study in China. 61
31179102 2019
8
Thymic neoplasms patients complicated with bronchiectasis: Case series in a Chinese hospital and literature review. 61
30758142 2019
9
Precursor B-cell development in bone marrow of Good syndrome patients. 61
30690192 2019
10
Aplastic Anemia and Good Syndrome in a Heavily Treated Stage IV Thymoma Patient: A Case Report and Review of the Literature. 61
31781423 2019
11
Humoral immunodeficiencies: conferred risk of infections and benefits of immunoglobulin replacement therapy. 61
30536429 2018
12
Agranulocytosis and Good Syndrome in Patient with Thymoma-The Role of Immunosuppressive Treatment after Thymectomy. 61
30166018 2018
13
Prevention of infectious diseases in patients with Good syndrome. 61
29878906 2018
14
Severe diarrhoea due to Cystoisospora belli infection in a Good syndrome patient. 61
29625163 2018
15
Good syndrome: immunodeficiency associated with thymoma. 61
29984501 2018
16
Delayed Diagnosis and Complications of Predominantly Antibody Deficiencies in a Cohort of Australian Adults. 61
29867917 2018
17
Atypical VZV Retinitis in a Patient with Good Syndrome. 61
27494574 2018
18
Aberrant Peripheral Immune Function in a Good Syndrome Patient. 61
29850635 2018
19
Cytomegalovirus Retinitis as the First Manifestation of Good Syndrome. 61
27715373 2018
20
Thymoma-associated multi-organ autoimmunity: A case of graft-versus-host disease-like erythroderma complicated by Good syndrome successfully treated by thymectomy. 61
28256733 2017
21
Thymoma with immunodeficiency/Good syndrome associated with myasthenia gravis. 61
28450687 2017
22
[Good's syndrome and congenital toxoplasmosis due to maternal reactivation during pregnancy]. 61
28347510 2017
23
Ecthyma gangrenosum without bacteraemia: evidence in favour of a broader definition. 61
28239902 2017
24
Pure Red Cell Aplasia Associated with Good Syndrome. 61
28382272 2017
25
[Acquired hypogammaglobulinemia associated with thymoma: Good syndrome]. 61
29881497 2017
26
Good syndrome and other causes of cytomegalovirus retinitis in HIV-negative patients-case report and comprehensive review of the literature. 61
26809342 2016
27
Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective. 61
27497628 2016
28
Tuberculous lymphadenitis in a patient with Good syndrome. 61
27487062 2016
29
Thymic Epithelial Tumor-Associated Cytopenia: A 10-Year Observational Study in France. 61
26768832 2016
30
Good syndrome presenting with CD8⁺ T-Cell large granular lymphocyte leukemia. 61
26429871 2015
31
Good syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complications. 61
25828999 2015
32
Cytokine profiles contribute to understanding the pathogenic difference between Good syndrome and oral lichen planus: two case reports and literature review. 61
25860215 2015
33
A case of good syndrome presumed secondary to metastatic pancreatic thymoma in a patient presenting with a myasthenic crisis postthymectomy. 61
25695921 2015
34
Three difficult cases: the challenge of autoimmunity, immunodeficiency and recurrent infections in patients with Good syndrome. 61
25059810 2015
35
Good syndrome and lichen planus: case and review. 61
24588858 2014
36
Good syndrome and polymyositis. 61
24685393 2014
37
Good syndrome, bad problem. 61
25426445 2014
38
Good syndrome, a rare disease that physicians cannot afford to overlook; case report and review of literature. 61
24397104 2013
39
Treatment of mucosal leishmaniasis (L. infantum) with miltefosine in a patient with Good syndrome. 61
23871798 2013
40
Good Syndrome, a rare cause of refractory chronic diarrhea and recurrent pneumonia in a Chinese patient after thymectomy. 61
23697574 2013
41
Thymoma and immunodeficiency: (18)F-FDG-PET/CT imaging of Good syndrome. 61
23687646 2013
42
Autoimmune retinopathy in benign thymoma after good syndrome-associated cytomegalovirus retinitis. 61
23323587 2013
43
TACI mutation p.Lys154Ter identified in Good Syndrome. 61
23165285 2013
44
[Association of Good syndrome with pure red cell aplasia: report of one case]. 61
23282780 2012
45
A case of Good syndrome with refractory gastrointestinal ulcers. 61
22715016 2012
46
A case of Good syndrome with pulmonary lesions similar to diffuse panbronchiolitis. 61
22576393 2012
47
[A Good syndrome associated with pure red cell aplasia]. 61
22073609 2011
48
[Glossitis by mixed infection of cytomegalovirus and herpes simplex virus during therapy for pure red cell aplasia complicated with Good syndrome]. 61
21897079 2011
49
Diagnostic strategy for patients with hypogammaglobulinemia in rheumatology. 61
21036646 2011
50
[Antibody deficiencies. A survey from three Clinical Immunology Centers for adults in Buenos Aires city]. 61
21893448 2011

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:

(show all 12)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.2 TNFRSF1B TNFRSF13B TNF GNG10 EPHB6 CD40LG
2
Show member pathways
12.62 TNFRSF13B TNF CD8A CD40LG CD4
3
Show member pathways
12.41 TNFRSF1B TNF GNG10 CD40LG CD4
4
Show member pathways
12.36 TNFRSF1B TNFRSF13B TNF GNG10 CD40LG
5 12.32 TNFRSF1B TNF CD8A CD40LG CD19
6 11.73 TNF CD8A CD4 CD19
7 11.62 TNF CD8A CD4 CD19
8 11.57 TNF CD8A CD4
9 11.43 TNF CD8A CD4 CD19
10
Show member pathways
11.32 TNFRSF1B TNFRSF13B TNF CD40LG
11 10.98 TNFRSF13B CD40LG CD4 CD19
12 10.6 TNFRSF13B CD8A CD40LG CD4 CD19

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 9.7 TNFRSF13B TNF EPHB6 CD8A CD40LG CD4
2 membrane raft GO:0045121 9.26 TNFRSF1B TNF CD4 CD19
3 external side of plasma membrane GO:0009897 9.1 TNF RAET1G CD8A CD40LG CD4 CD19

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

# Name GO ID Score Top Affiliating Genes
1 cell surface receptor signaling pathway GO:0007166 9.62 TNFRSF1B TNFRSF13B CD8A CD4
2 T cell mediated immunity GO:0002456 9.37 EPHB6 CD8A
3 negative regulation of lipid storage GO:0010888 9.26 TNF CRP
4 tumor necrosis factor-mediated signaling pathway GO:0033209 9.26 TNFRSF1B TNFRSF13B TNF CD40LG
5 regulation of immunoglobulin secretion GO:0051023 9.16 TNF CD40LG
6 immune response GO:0006955 9.1 TNFRSF1B TNF RAET1G CD8A CD40LG 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 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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