Thymoma and Hypogammaglobulinemia (Good’s Syndrome)

Brasher, George W.; Howard, Paxton H.; Brindley, G. V.

doi:10.1016/s0039-6109(16)39697-9

Cited by 7 publications

(3 citation statements)

References 15 publications

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“…Reasons for this association are obscure and, since sev eral mechanisms including decreased B or T cell helper function or increased T suppres sor activity might be involved, an interpre tation of possible underlying defects cannot be made. It may be of interest that autoim mune diseases, of which alopecia areata may be one, are frequently associated with hypogammaglobulinemia and that humoral substances have been implicated in the de pressed function of immunoglobulin-pro ducing cells [3].…”

Section: Discussionmentioning

confidence: 99%

Immunological Studies in Patients with Alopecia Areata

Herzer¹,

Czarnetzki²,

Holzmann³

et al. 1979

Int Arch Allergy Immunol

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Cellular and humoral immune parameters were studied in 10 patients with alopecia areata totalis and universalis. Trends to low normal immunoglobulin levels and in vitro lymphocyte transformation to concanavalin A were observed. Serum CH-50 levels and skin test responses to mumps, Candida, trichophytin and dinitrochlorobenzene (DNCB) were normal, as were the numbers of T and B cells. Immunosuppressive serum factors were not found in vitro. 1 patient with alopecia universalis of 33 years duration developed hair at the site of DNCB skin testing. The findings point to rather subtle immune defects in patients with this disease.

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Section: Discussionmentioning

confidence: 99%

Immunological Studies in Patients with Alopecia Areata

Herzer¹,

Czarnetzki²,

Holzmann³

et al. 1979

Int Arch Allergy Immunol

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“…The heterogeneity of both the clinical presentation and the immunological abnormalities of Good’s syndrome has been repeatedly underlined (see Table 4 ). In early reports [ 12 ] with only 30 cases published, the picture was dominated by sinopulmonary infections and hypogammaglobulinemia, with mention of the rarity of pure red cell aplasia or pancytopenia, and other features such as chronic diarrhea with malabsorption and also fungal infections. The underlying mechanism of these symptoms was unknown, since at the time the precise role of the thymus in the adult immune system had not been adequately defined [ 12 ], but the presence of defects of delayed hypersensitivity was noted.…”

Section: Discussionmentioning

confidence: 99%

“…It was well known that myasthenia gravis was associated with a thymoma since 1950 [ 11 ], and at the time of R.A. Good’s description, nothing could link this thymic neoplasia to agammaglobulinemia. This association was rapidly known as Good’s syndrome [ 12 ], and several case and series reports have been published until today: we retrieved 154 papers with “Good’s syndrome” in their title in Pub Med. Despite being diagnosed mainly in adults (with median age at diagnosis 55–64 years in a recent review [ 13 ]), it was included in the primary immune deficiencies, with the name of Immunodeficiency with thymoma (sometimes changed to thymoma with immunodeficiency) [ 14 , 15 , 16 ], which is its official designation.…”

Section: Introductionmentioning

confidence: 99%

Insights from a Case of Good’s Syndrome (Immunodeficiency with Thymoma)

Paganelli

Lizia²,

D'Urbano

et al. 2023

Biomedicines

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Immunodeficiency with thymoma was described by R.A. Good in 1954 and is also named after him. The syndrome is characterized by hypogammaglobulinemia associated with thymoma and recurrent infections, bacterial but also viral, fungal and parasitic. Autoimmune diseases, mainly pure red cell aplasia, other hematological disorders and erosive lichen planus are a common finding. We describe here a typical case exhibiting all these clinical features and report a detailed immunophenotypic assessment, as well as the positivity for autoantibodies against three cytokines (IFN-alpha, IL-6 and GM-CSF), which may add to known immune abnormalities. A review of the published literature, based on case series and immunological studies, offers some hints on the still unsolved issues of this rare condition.

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Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome)

Watts

Kelly

1990

Med. Pediatr. Oncol.

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An 8-year-old male underwent excision of an encapsulated thymoma. Four months later he presented with disseminated, fatal varicella. Evaluation of his immune status during the terminal illness revealed hypogammaglobulinemia and lymphopenia consistent with a diagnosis of Good's syndrome (immunodeficiency with thymoma). This is the first case of Good's syndrome reported in a child and the first case of fatal varicella associated with Good's syndrome. The combination of this rare pediatric tumor and immunodeficiency is discussed. Despite specific antiviral therapy, varicella remains a deadly disease in the immunocompromised host.

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Thymoma and Hypogammaglobulinemia (Good’s Syndrome)

Cited by 7 publications

References 15 publications

Immunological Studies in Patients with Alopecia Areata

Immunological Studies in Patients with Alopecia Areata

Insights from a Case of Good’s Syndrome (Immunodeficiency with Thymoma)

Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome)

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