Rare Diseases Referral Center, Internal Medicine 1, Ca' Foncello Hospital, AULSS2 Marca Trevigiana, Department of Medicine - DIMED, University of Padova, Treviso, Italy.
Hospital de Santa Maria, Lisbon, Portugal.
Expert Rev Hematol. 2023 Apr;16(4):237-243. doi: 10.1080/17474086.2023.2176843. Epub 2023 Apr 3.
Secondary antibody deficiencies (SAD) are often a side effect of specific therapies that target B cells directly or affect the antibody response indirectly. Treatment of immunodeficiency by immunoglobulin replacement therapy (IgRT) is well established in primary antibody deficiencies, although the evidence for its use in SAD is less well established. To fill the gap and provide opinion and advice for daily practice, a group of experts met to discuss current issues and share best practical experience.
A total of 16 questions were considered that covered use of a tailored approach, definition of severe infections, measurement of IgG levels and specific antibodies, indications for IgRT, dosage, monitoring, discontinuation of IgRT, and Covid-19.
Key points for better management SID should include characterization of the immunological deficiency, determination of the severity and degree of impairment of antibody production, distinguish between primary and secondary deficiency, and design a tailored treatment protocol that should include dose, route, and frequency of Ig replacement. There remains the need to carry out well-designed clinical studies to develop clear guidelines for the use of IgRT in patients with SAD.
继发性抗体缺陷(SAD)通常是直接针对 B 细胞的特定疗法或间接影响抗体反应的副作用。免疫球蛋白替代疗法(IgRT)在原发性抗体缺陷中的治疗已得到充分确立,尽管其在 SAD 中的应用证据尚不充分。为了填补这一空白,并为日常实践提供意见和建议,一组专家开会讨论了当前的问题,并分享了最佳的实践经验。
共考虑了 16 个问题,涵盖了量身定制方法的使用、严重感染的定义、IgG 水平和特定抗体的测量、IgRT 的适应证、剂量、监测、IgRT 的停药以及新冠病毒。
更好地管理 SID 的要点应包括免疫缺陷的特征描述、确定抗体产生的严重程度和程度、区分原发性和继发性缺陷、以及设计量身定制的治疗方案,其中应包括 Ig 替代的剂量、途径和频率。仍有必要开展精心设计的临床研究,为 SAD 患者使用 IgRT 制定明确的指南。