Hypogammaglobulinemia - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Hypogammaglobulinemia can be primary (congenital) or secondary. It can present in childhood and in adults, and can affect both sexes.
Primary hypogammaglobulinemia may have a delay of several years between clinical presentation and diagnosis. The most common cause is common variable immunodeficiency.
Causes of secondary hypogammaglobulinemia include gastrointestinal losses (e.g., malabsorption/protein-losing enteropathy), nephrotic syndrome, hematologic malignancy, and medication (e.g., immunosuppressives, such as corticosteroids and chemotherapy).
Patients can be predisposed to recurrent upper and lower respiratory tract infections.
Treatment is tailored to specific causes and the clinical presentation of each patient, and includes intravenous or subcutaneous immunoglobulin replacement.

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Definition

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Citations

Key Articles

Tangye SG, Al-Herz W, Bousfiha A, et al. Human inborn errors of immunity: 2022 update on the classification from the International Union of Immunological Societies Expert Committee J Clin Immunol. 2022 Jun 24;1-35 [Epub ahead of print].[Abstract][Full Text]
Otani IM, Lehman HK, Jongco AM, et al. Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: a Work Group report of the AAAAI Primary Immunodeficiency and Altered Immune Response Committees. J Allergy Clin Immunol. 2022 May;149(5):1525-60.[Abstract][Full Text]
Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015 Nov;136(5):1186-205;e1-78.[Abstract][Full Text]
Kohn DB, Hershfield MS, Puck JM, et al. Consensus approach for the management of severe combined immune deficiency caused by adenosine deaminase deficiency. J Allergy Clin Immunol. 2019 Mar;143(3):852-63.[Abstract][Full Text]
National Blood Authority, Australia. Criteria for the clinical use of immunoglobulin in Australia: version 3. 2018 [internet publication].[Full Text]

Other Online Resources

European Society for Immunodeficiencies: diagnostic criteria for PID
INFO4PI.org: 10 warning signs of primary immunodeficiency

Referenced Articles

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2. Otani IM, Lehman HK, Jongco AM, et al. Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: a Work Group report of the AAAAI Primary Immunodeficiency and Altered Immune Response Committees. J Allergy Clin Immunol. 2022 May;149(5):1525-60.[Abstract][Full Text]
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