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Good's syndrome

Related gene: Unknown

Inheritance pattern: Unknown

This primary immunodeficiency is characterized by low immunoglobulins together with a thymoma (tumor on the thymus). Good’s syndrome is usually first suspected when a thymic tumor is seen on a chest X-ray, although in about half the cases the history of recurrent infections precedes the detection of the thymoma. Most patients are adults. Removal of the thymoma does not cure the immunodeficiency, although it may help other symptoms. Eosinophils may be very low or undetectable in these patients.

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Other antibody deficiencies 

Similar to the patients with X-linked agammaglobulinemia (XLA)hyper IgM syndromecommon variable immune deficiency (CVID), and specific antibody deficiency (SAD), individuals with less common antibody deficiencies usually present with upper respiratory infections or infections of the sinuses or lungs, typically with organisms like Streptococcus pneumoniae and Haemophilus influenzae. Laboratory studies show low immunoglobulins and/or deficient specific antibody production. Many of these disorders also include abnormalities in the cells responsible for generating or maintaining an antibody response. The patients often improve with antibiotics but get sick again when these are discontinued. The cornerstone of therapy for antibody deficiency disorders is immunoglobulin (Ig) replacement therapy.

This page contains general medical and/or legal information that cannot be applied safely to any individual case. Medical and/or legal knowledge and practice can change rapidly. Therefore, this page should not be used as a substitute for professional medical and/or legal advice.

Adapted from the IDF Patient & Family Handbook for Primary Immunodeficiency Diseases, Sixth Edition 
Copyright ©2019 by Immune Deficiency Foundation, USA