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Lifelong immunoglobulin replacement is not always necessary: A case description of a patient with recurrent infections and hypogammaglobulinemia.
International Journal of Immunopathology and Pharmacology ( IF 3.5 ) Pub Date : 2019-04-11 , DOI: 10.1177/2058738419843364
Katarzyna Napiorkowska-Baran 1 , Radoslaw Janicki 2 , Sylwia Koltan 3 , Ewa Szynkiewicz 4 , Zbigniew Bartuzi 5
Affiliation  

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.

中文翻译:

并非总是需要终身更换免疫球蛋白:反复感染和低血球蛋白血症的患者的病例描述。

伴有感染的体液免疫缺陷是人免疫球蛋白替代疗法的指征。治疗是终生的还是必要的只是暂时的,取决于缺乏的性质:主要(持续)或继发(持续或短暂)。区分原发性和继发性免疫缺陷并不总是那么容易,尤其是在成年人中。这篇文章介绍了一名39岁患者的病历,其医学检查结果提示原发性体​​液免疫缺陷。当患者怀孕时,首次在38岁时诊断出缺乏症。该患者符合免疫球蛋白G替代疗法的条件。临床改善。怀孕结束后,观察到免疫学指标的系统性改善,建议解决免疫缺陷。决定终止免疫球蛋白替代。由于对疫苗有反应能力,在诊断过程中已得到确认,因此建议使用预防性疫苗。没有发生严重感染的复发。该临床过程最终使继发免疫缺陷的诊断成为可能。所呈现的病例显示了积极方法对诊断和治疗过程,不断评估临床过程,监测IgG浓度的重要性,以及认识到在我们没有遗传确认该疾病的情况下,诊断可能更改。没有发生严重感染的复发。该临床过程最终使继发免疫缺陷的诊断成为可能。所呈示的病例显示了一种积极方法对诊断和治疗过程,不断评估临床过程,监测IgG浓度的重要性,以及认识到在我们没有遗传证实该疾病的情况下,诊断可能更改。没有发生严重感染的复发。该临床过程最终使继发免疫缺陷的诊断成为可能。所呈示的病例显示了一种积极方法对诊断和治疗过程,不断评估临床过程,监测IgG浓度的重要性,以及认识到在我们没有遗传证实该疾病的情况下,诊断可能更改。
更新日期:2019-11-01
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