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Unspecified hypogammaglobulinemia

Related gene: Unknown

Inheritance pattern: Unknown

Unspecified hypogammaglobulinemia, also known as unspecified antibody deficiency or idiopathic primary hypogammaglobulinemia, describes an individual with one or more deficiencies of IgG, IgA and/or IgM serum immunoglobulins who does not meet the diagnostic criteria for other antibody deficiencies. In general, these patients do not have an impaired ability to produce adequate levels of antibodies against vaccine antigens. In some patients, unspecified hypogammaglobulinemia may simply be a physiologic variant without any clinical significance. However, it may indicate a developing immunodeficiency and should be monitored, particularly if the patient begins to develop frequent and/or severe infections.

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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 
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