1 Jan Biziel University Hospital No. 2, Ludwik Rydygier Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland.
2 Hospital of Ministry of the Interior and Administration, Bydgoszcz, Poland.
Int J Immunopathol Pharmacol. 2019 Jan-Dec;33:2058738419843364. doi: 10.1177/2058738419843364.
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.
伴有感染的体液免疫缺陷是人类免疫球蛋白替代治疗的指征。治疗是终身的还是仅暂时需要取决于缺陷的性质:原发性(持续性)或继发性(持续性或一过性)。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。本文介绍了一例 39 岁患者的病史和医学检查结果,这些结果提示存在原发性体液免疫缺陷。该缺陷首次在 38 岁时被诊断,当时患者怀孕。患者有资格接受免疫球蛋白 G 替代治疗。临床状况得到改善。妊娠结束后,观察到免疫参数的系统性改善,提示免疫缺陷得到解决。决定停止免疫球蛋白替代治疗。由于在诊断过程中确认了对疫苗的反应能力,建议接种预防疫苗。没有再次发生严重感染。最终的临床过程使我们能够做出继发性免疫缺陷的诊断。所介绍的病例表明积极进行诊断和治疗过程、持续评估临床过程、监测 IgG 浓度的重要性,以及在没有疾病遗传确认的情况下,诊断可能会发生变化的认识。