Very rare. Acquired immunodepression syndrome associated with thymomas. It combines hypogammaglobulinemia, leukopenia affecting mainly B lymphocytes (virtual absence of circulating B lymphocytes) and cellular immunodeficiency.

Clinical presentation :

-         usual onset between the 40 and 50 years of age, but pediatric cases have been described.

-        hypogammaglobulinemia that does not correct by thymectomy

-        frequent infections (95 %): bacterial (especially bronchopulmonary and rhinosinusal), viral and fungal. These recurrent respiratory infections can cause bronchial bronchiectasis.

-        autoimmune disease (76 %), which may require immunosuppressive treatment: oral lichen planus, colitis, bone marrow aplasia limited to red blood cells, myasthenia gravis.

-        the diagnosis of thymoma often precedes that of immunodepression.

-        prognosis is poor.

Anesthetic implications:

immunodepression, bronchiectasies

References

-        Watts RG, Kelly DR.
Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome).
Med Pediatr Oncol 1990;18 :246-51

-        Lee SH, Lee SM, Yang SC, Yoo CG, Kim YW, Shim YS, Han SK.
A case of granulomatous lung disease in a patient with Good's syndrome.
Korean J Intern Med 2008;23 :219-22.

-        Paranavitane S, Handagala S, De Silva R, Chang T.
Thymoma complicated with myasthenia gravis and Good syndrome-a therapeutic conundrum: a case report.
Journal of Medical Case Reports 2019 ; 13:348 doi.org/10.1186/s13256-019-2289-z

-        Malphettes M, Gérard L, Galicier L, Boutboul D,  Asli B, Szalat R, Perlat A et al.
Good Syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complication.
Clinical Infectious Diseases 2015 ; 61 : e13-e19,

Updated: January 2024