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Lifelong immunoglobulin replacement is not always necessary: A case description of a patient with recurrent infections and hypogammaglobulinemia

Humoral immunodeficiency with accompanying infections is an indication for human immunoglobulin replacement therapy. Whether treatment will be lifelong or necessary only temporarily depends on the nature of deficiency: primary (persistent) or secondary (persistent or transient). It is not always eas...

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Autores principales: Napiorkowska-Baran, Katarzyna, Janicki, Radoslaw, Koltan, Sylwia, Szynkiewicz, Ewa, Bartuzi, Zbigniew
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6458662/
https://www.ncbi.nlm.nih.gov/pubmed/30968707
http://dx.doi.org/10.1177/2058738419843364
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author Napiorkowska-Baran, Katarzyna
Janicki, Radoslaw
Koltan, Sylwia
Szynkiewicz, Ewa
Bartuzi, Zbigniew
author_facet Napiorkowska-Baran, Katarzyna
Janicki, Radoslaw
Koltan, Sylwia
Szynkiewicz, Ewa
Bartuzi, Zbigniew
author_sort Napiorkowska-Baran, Katarzyna
collection PubMed
description Humoral immunodeficiency with accompanying infections is an indication for human immunoglobulin replacement therapy. Whether treatment will be lifelong or necessary only temporarily depends on the nature of deficiency: primary (persistent) or secondary (persistent or transient). It is not always easy to distinguish between primary and secondary immunodeficiency, especially in adults. The article presents a case of a 39-year-old patient with anamnesis and medical tests results that suggested primary humoral immunodeficiency. The deficiency was diagnosed for the first time at the age of 38, when the patient was pregnant. The patient was qualified for immunoglobulin G replacement therapy. Clinical improvement was achieved. After the end of pregnancy, systematic improvement in immunological parameters was observed, suggesting the resolution of immunodeficiency. A decision was made to discontinue immunoglobulin replacement. Due to the ability to respond to vaccine, confirmed during diagnosis, preventive vaccines were recommended. There was no recurrence of serious infections. The clinical course finally enabled a diagnosis of secondary immunodeficiency. The presented case shows the importance of an active approach to the diagnostic and therapeutic process, constant assessment of clinical course, monitoring of IgG concentrations, and the awareness that in the situation when we do not have a genetic confirmation of the disease, the diagnosis may change.
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spelling pubmed-64586622019-04-19 Lifelong immunoglobulin replacement is not always necessary: A case description of a patient with recurrent infections and hypogammaglobulinemia Napiorkowska-Baran, Katarzyna Janicki, Radoslaw Koltan, Sylwia Szynkiewicz, Ewa Bartuzi, Zbigniew Int J Immunopathol Pharmacol Letter to the Editor Humoral immunodeficiency with accompanying infections is an indication for human immunoglobulin replacement therapy. Whether treatment will be lifelong or necessary only temporarily depends on the nature of deficiency: primary (persistent) or secondary (persistent or transient). It is not always easy to distinguish between primary and secondary immunodeficiency, especially in adults. The article presents a case of a 39-year-old patient with anamnesis and medical tests results that suggested primary humoral immunodeficiency. The deficiency was diagnosed for the first time at the age of 38, when the patient was pregnant. The patient was qualified for immunoglobulin G replacement therapy. Clinical improvement was achieved. After the end of pregnancy, systematic improvement in immunological parameters was observed, suggesting the resolution of immunodeficiency. A decision was made to discontinue immunoglobulin replacement. Due to the ability to respond to vaccine, confirmed during diagnosis, preventive vaccines were recommended. There was no recurrence of serious infections. The clinical course finally enabled a diagnosis of secondary immunodeficiency. The presented case shows the importance of an active approach to the diagnostic and therapeutic process, constant assessment of clinical course, monitoring of IgG concentrations, and the awareness that in the situation when we do not have a genetic confirmation of the disease, the diagnosis may change. SAGE Publications 2019-04-10 /pmc/articles/PMC6458662/ /pubmed/30968707 http://dx.doi.org/10.1177/2058738419843364 Text en © The Author(s) 2019 http://www.creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Letter to the Editor
Napiorkowska-Baran, Katarzyna
Janicki, Radoslaw
Koltan, Sylwia
Szynkiewicz, Ewa
Bartuzi, Zbigniew
Lifelong immunoglobulin replacement is not always necessary: A case description of a patient with recurrent infections and hypogammaglobulinemia
title Lifelong immunoglobulin replacement is not always necessary: A case description of a patient with recurrent infections and hypogammaglobulinemia
title_full Lifelong immunoglobulin replacement is not always necessary: A case description of a patient with recurrent infections and hypogammaglobulinemia
title_fullStr Lifelong immunoglobulin replacement is not always necessary: A case description of a patient with recurrent infections and hypogammaglobulinemia
title_full_unstemmed Lifelong immunoglobulin replacement is not always necessary: A case description of a patient with recurrent infections and hypogammaglobulinemia
title_short Lifelong immunoglobulin replacement is not always necessary: A case description of a patient with recurrent infections and hypogammaglobulinemia
title_sort lifelong immunoglobulin replacement is not always necessary: a case description of a patient with recurrent infections and hypogammaglobulinemia
topic Letter to the Editor
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6458662/
https://www.ncbi.nlm.nih.gov/pubmed/30968707
http://dx.doi.org/10.1177/2058738419843364
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