Immunodeficiency and thymoma in Good syndrome: Two sides of the same coin

Guevara-Hoyer, Kissy; Fuentes-Antrás, Jesús; Gastardi, Joaquín Calatayud; Sánchez-Ramón, Silvia

doi:10.1016/j.imlet.2020.12.010

Cited by 22 publications

(48 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Treatment of this disease usually involves resection of the thymoma, proper targeted antibiotic therapy in patients with underlying infection, and a thorough immunological workup. In patients with global hypogammaglobulinemia, IVIG is indicated [4][5]14]. Our patient posed a further complication, as he developed a severe adverse reaction to the first dose of IVIG.…”

Section: Discussionmentioning

confidence: 86%

“…While the pathophysiology of immunodeficiency remains elusive, there have been some proposed hypotheses. In a recent study by Guevara et al, it was hypothesized that the underlying mechanism of Good's syndrome may be due to the improper differentiation of plasma cells, loss of naive memory of the CD4+ T cell population that may lead to defects in cell-mediated immunity and increase susceptibility to infections and anti-cytokine antibodies (mainly interferon (IFN) α, IFN-β, interleukin (IL) 1α, IL-12, IL-17A) that may have a negative effect on the growth and differentiation of pro B lymphocytes [14].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Achromobacter xylosoxidans/denitrificans Bacteremia in a Patient With Good’s Syndrome

Choudhury

Papineni

Ramachandruni

et al. 2021

Cureus

View full text Add to dashboard Cite

Good's syndrome is a rare, adult-onset immunodeficiency syndrome associated with thymomas. Here, we present a 71-year-old, previously healthy male with a history of opportunistic infections status postthymectomy, who presented with a septic knee complicated with bacteremia without any inciting factor. Therefore, a diagnosis of Good syndrome was made. While this is a rare disease, clinicians should have a high suspicion in patients with a history of thymoma. Early diagnosis and treatment can reduce opportunistic infections and improve morbidity and mortality.

show abstract

Section: Discussionmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Achromobacter xylosoxidans/denitrificans Bacteremia in a Patient With Good’s Syndrome

Choudhury

Papineni

Ramachandruni

et al. 2021

Cureus

View full text Add to dashboard Cite

show abstract

“…GS is a rare adult-onset combined immunodeficiency characterized by thymoma and hypogammaglobulinaemia [1] . Most cases occur between the ages of 40 and 70, with equal distribution between the sexes [2] . Although it was first described in 1954, its pathogenesis remains unclear.…”

Section: Discussionmentioning

confidence: 99%

“…Although it was first described in 1954, its pathogenesis remains unclear. Immunological disturbances may include low to absent circulating B-lymphocytes, hypogammaglobulinaemia, decreased T-lymphocytes, inversion of the CD4+/CD8+ ratio, and functional defects in cellmediated immunity [2,3] . The phenotype is variable.…”

Section: Discussionmentioning

confidence: 99%

A Case of Severe COVID-19 in a Patient with Good's Syndrome

Duarte

Faria

Patronillo

et al. 2021

European Journal of Case Reports in Internal Medicine

View full text Add to dashboard Cite

Good’s syndrome is a rare adult-onset combined immunodeficiency. The association of hypogammaglobulinaemia with a history of recurrent infectious or autoimmune manifestations in a middle-aged patient with evidence of a mediastinal mass should lead to the clinical suspicion of Good’s syndrome. The mortality rate associated with infectious complications is high. Thus, although it is rare, the disease should be diagnosed early so that proper treatment can be started. Thymectomy and immunoglobulin replacement are the main therapeutic strategies. We describe the case of a patient with a history of thymoma and recurrent respiratory infections, with a late diagnosis of Good’s syndrome in the context of severe organizing pneumonia secondary to COVID-19.

show abstract

“…Thymoma-associated immunodeficiency is known as Good's syndrome and includes hypogammaglobulinaemia, decreased or absent B lymphocytes, CD4 + /CD8 + inversion and decreased T lymphocytes. In addition, autoimmune manifestations may occur 4 . The etiology of thymoma related PWCA is still unknown, but it appears to have an autoimmune background.…”

Section: Discussionmentioning

confidence: 99%