Clinicopathologic features of Good's syndrome: Two cases and literature review.
Good’s syndrome
common variable immunodeficiency
diarrhea
immunodeficiency disease
opportunistic infection
thymoma
Journal
Open medicine (Warsaw, Poland)
ISSN: 2391-5463
Titre abrégé: Open Med (Wars)
Pays: Poland
ID NLM: 101672167
Informations de publication
Date de publication:
2021
2021
Historique:
received:
23
11
2020
revised:
16
02
2021
accepted:
24
02
2021
entrez:
14
4
2021
pubmed:
15
4
2021
medline:
15
4
2021
Statut:
epublish
Résumé
Good's syndrome (GS) is an immunodeficiency disease, causing thymoma, low or absent B-cells, hypogammaglobulinemia, and defects in cell-mediated immunity. The most common clinical presentation is recurrent infection, followed by refractory diarrhea, due to the immunodeficiency. However, there are only few reports on intestinal endoscopy and pathology. We report here two typical GS cases with diarrhea as the prominent manifestation. Both cases presented with thymoma combined with immunodeficiency, characterized by hypogammaglobulinemia, low or absent B lymphocytes, and decreased T-cells with inverted CD4 GS should be considered among the differential diagnoses for patients with unexplained recurrent diarrhea and opportunistic infection. Although it was regarded as a subset of CVID with thymoma, GS had a different clinical-pathological feature from CVID.
Sections du résumé
BACKGROUND
BACKGROUND
Good's syndrome (GS) is an immunodeficiency disease, causing thymoma, low or absent B-cells, hypogammaglobulinemia, and defects in cell-mediated immunity. The most common clinical presentation is recurrent infection, followed by refractory diarrhea, due to the immunodeficiency. However, there are only few reports on intestinal endoscopy and pathology.
CASE SUMMARY
METHODS
We report here two typical GS cases with diarrhea as the prominent manifestation. Both cases presented with thymoma combined with immunodeficiency, characterized by hypogammaglobulinemia, low or absent B lymphocytes, and decreased T-cells with inverted CD4
CONCLUSION
CONCLUSIONS
GS should be considered among the differential diagnoses for patients with unexplained recurrent diarrhea and opportunistic infection. Although it was regarded as a subset of CVID with thymoma, GS had a different clinical-pathological feature from CVID.
Identifiants
pubmed: 33851032
doi: 10.1515/med-2021-0256
pii: med-2021-0256
pmc: PMC8019250
doi:
Types de publication
Case Reports
Langues
eng
Pagination
532-539Informations de copyright
© 2021 Yi-Dan Chen et al., published by De Gruyter.
Déclaration de conflit d'intérêts
Conflict of interest: The authors state no conflict of interest.
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