The Natural History of Untreated Primary Hypogammaglobulinemia in Adults: Implications for the Diagnosis and Treatment of Common Variable Immunodeficiency Disorders (CVID)

Frontiers in Immunology
Rohan AmeratungaSee-Tarn Woon

Abstract

Background: Adults with primary hypogammaglobulinemia are frequently encountered by clinicians. Where IgG levels are markedly decreased, most patients are treated with subcutaneous or intravenous immunoglobulin (SCIG/IVIG), because of the presumed risk of severe infections. The natural history of untreated severe asymptomatic hypogammaglobulinemia is thus unknown. Similarly, there are no long-term prospective studies examining the natural history of patients with moderate reductions in IgG. Methods: In 2006, we began a prospective cohort study of patients with symptomatic and asymptomatic reductions in IgG who were not immediately commenced on SCIG/IVIG. Over the course of 12 years, 120 patients were enrolled in the NZ hypogammaglobulinemia study (NZHS) including 59 who were asymptomatic. Results: Five patients with profound primary hypogammaglobulinemia (IgG < 3 g/l), who were not on regular SCIG/IVIG have remained well for a mean duration of 139 months. This study has also shown most asymptomatic patients with moderate hypogammaglobulinemia (IgG 3.0-6.9 g/l) have been in good health for a mean observation period of 96 months. We have only identified one asymptomatic patient with moderate hypogammaglobulinemia who experienced ...Continue Reading

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Citations

Nov 14, 2019·Clinical Reviews in Allergy & Immunology·Rohan Ameratunga, See-Tarn Woon
Jul 2, 2019·Clinical and Experimental Immunology·R AmeratungaS-T Woon
Dec 13, 2019·Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology·Rohan AmeratungaHilary Blacklock
Jan 22, 2021·Allergy, Asthma & Immunology Research·Joo Hee KimHae Sim Park
Jul 9, 2021·Clinical Reviews in Allergy & Immunology·Rohan AmeratungaSee-Tarn Woon
Jun 29, 2021·The Journal of Allergy and Clinical Immunology. in Practice·Rohan AmeratungaSee-Tarn Woon
Sep 18, 2021·Expert Review of Clinical Immunology·Rohan AmeratungaAnna E S Brooks
Oct 19, 2021·Frontiers in Immunology·Anette Tarp HansenJens Magnus Bernth Jensen

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Methods Mentioned

BETA
exome sequencing

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