Thymoma with immunodeficiency (Good's syndrome): Review of the literature apropos three cases

@article{Miyakis2006ThymomaWI,
  title={Thymoma with immunodeficiency (Good's syndrome): Review of the literature apropos three cases},
  author={Spiros Miyakis and Angelos V Pefanis and Freda H. Passam and George R Christodulakis and Paraskevi A Roussou and Theodore D. Mountokalakis},
  journal={Scandinavian Journal of Infectious Diseases},
  year={2006},
  volume={38},
  pages={314 - 320}
}
Good's syndrome is the association of thymoma with immunodeficiency, characterized by hypogammaglobulinaemia, depleted B-cells, diminished T-cells and inversion of the CD4/CD8 ratio. The initial clinical presentation is either with a mass lesion-thymoma that is usually benign, or with recurrent infections due to immunodeficiency. Thymectomy usually favourably affects associated conditions, such as pure red cell aplasia, but does not improve hypogammaglobulinaemia, thus the patient remains… 

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References

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TLDR
The cause and pathogenesis of this disorder are unknown, although there is some evidence that the basic defect may be in the bone marrow (pre-B cell arrest, impaired maturation of erythroid and myeloid precursors in some patients).

Sweet's syndrome in a patient with idiopathic myelofibrosis and thymoma-myasthenia gravis-immunodeficiency complex: efficacy of treatment with etretinate.

TLDR
The patient's general condition unexpectedly improved, with long-lasting suppression of respiratory infections and a slight but consistent improvement of hematological parameters such as Hct, MCV, Hb level, RBC, WBC and lymphocyte absolute counts.

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

TLDR
Evaluation of his immune status during the terminal illness revealed hypogammaglobulinemia and lymphopenia consistent with a diagnosis of Good's syndrome (immunodeficiency with thymoma), the first case of Good’s syndrome reported in a child and the first cases of fatal varicella associated with Good's Syndrome.

Infections in Patients with Immunodeficiency with Thymoma (Good Syndrome): Report of 5 Cases and Review of the Literature

TLDR
Patients with GS present with a spectrum of sinopulmonary infections and pathogens similar to common variable immunodeficiency (CVID), and compared with patients with CVID, opportunistic infections appear to be more common in patients with GS, and patients withGS may have a worse prognosis.

Thymoma with immunodeficiency (Good's syndrome) associated with selective cobalamin malabsorption and benign IgM‐K gammopathy

Abstract. We have described the first case, to our knowledge, in which recurrent respiratory tract infections were the primary manifestation of thymoma with immunodeficiency (Good's syndrome)

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TLDR
A 47‐year‐old male presented to us in 1995 with diarrhoea, weight loss, lichen planus, oesophageal candidiasis and streptococcal pneumonia, and Good's syndrome (thymoma and hypogammaglobulinaemia) was suspected and confirmed by CT scans of the mediastinum.

Thymoma with immunodeficiency (Good's syndrome) associated with myasthenia gravis and benign IgG gammopathy.

TLDR
A case of thymoma with immunodeficiency associated with a rare combination of other parathymic syndromes including myasthenia gravis, benign IgG lambda M component, pernicious anemia, and diabetes is treated.

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TLDR
In this case, anemia improved in response to immunosuppressive agents as it did in most of the other cases, but an increased frequency of various infections was seen, since Good’s syndrome is an immunodeficiency condition.

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TLDR
B lymphopenia often occurred in this series of thymoma patients and was related to susceptibility to recurrent infections more than hypogammaglobulinemia, therefore, immunophenotype has to be monitored in follow-up of thye patients because it may reveal significant abnormalities.

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TLDR
There is particular concern that infection with mycoplasmas and enteroviruses can be resistant to treatment and the high incidence of lymphoma and gastric carcinoma in patients with common variable immunodeficiency is high-lighted.