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继发性抗体缺陷症患者皮下免疫球蛋白替代治疗:与原发性抗体缺陷症的真实世界证据比较。

Subcutaneous immunoglobulins replacement therapy in secondary antibody deficiencies: Real life evidence as compared to primary antibody deficiencies.

机构信息

Department of Medicine-DIMED, University of Padua, Padua, Italy.

Formerly Haematology and Clinical Immunology Unit, University of Padua, Padua, Italy.

出版信息

PLoS One. 2021 Mar 4;16(3):e0247717. doi: 10.1371/journal.pone.0247717. eCollection 2021.

DOI:10.1371/journal.pone.0247717
PMID:33661940
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7932095/
Abstract

Secondary antibody deficiencies (SAD) may require immunoglobulin replacement therapy (IgRT). While the intravenous route (IVIG) is broadly considered effective in SAD, the use of subcutaneous immunoglobulins (SCIG) is mainly adopted from the experience in primary antibody deficiencies (PAD), where SCIG have been shown to perform as effective as IVIG. However, evidence-based data on SCIG administration in SAD patients are still insufficient. Herein we retrospectively evaluated the efficacy and safety profile of SCIG treatment in 131 SAD patients as compared to a group of 102 PAD patients. We found SCIG being equally effective in reducing annual infectious rate both in SAD and PAD patients. However, SAD patients required lower SCIG dosage and lower IgG through level to achieve similar biological effect in terms of infection burden, at the steady state. SAD patients also showed better correlation between SCIG dose and serum IgG achieved value. Furthermore, within SAD, SCIG were found to work irrespective of the underlying disease. Especially in Non-Hodgkin Lymphoma patients, whose indication to IgRT is still not included in all guidelines and for whom evidence-based data are still lacking, SCIG were as effective as in Chronic Lymphocytic Leukemia or Multiple Myeloma patients, and SCIG discontinuation, without evidence of B cell recovery, led to IgG decline and relapsed infections. Finally, treatment tolerance in SAD patients was comparable to the PAD cohort. Globally, our data suggest that SCIG, as already appreciated in PAD, represent a valuable option in SAD patients, independent on the disease leading to antibody deficiency.

摘要

继发性抗体缺陷症(SAD)可能需要免疫球蛋白替代疗法(IgRT)。虽然静脉途径(IVIG)在 SAD 中被广泛认为是有效的,但皮下免疫球蛋白(SCIG)的使用主要是从原发性抗体缺陷症(PAD)的经验中得出的,其中已经证明 SCIG 与 IVIG 一样有效。然而,SAD 患者中 SCIG 给药的循证数据仍然不足。在此,我们回顾性评估了 131 例 SAD 患者和 102 例 PAD 患者使用 SCIG 治疗的疗效和安全性。我们发现 SCIG 在降低 SAD 和 PAD 患者的年感染率方面同样有效。然而,在达到相同的感染负担的生物效应时,SAD 患者需要更低的 SCIG 剂量和更低的 IgG trough 水平。SAD 患者还显示出 SCIG 剂量与达到的血清 IgG 值之间更好的相关性。此外,在 SAD 中,SCIG 被发现无论潜在疾病如何都有效。特别是在非霍奇金淋巴瘤患者中,IgRT 的适应证尚未被所有指南纳入,而且缺乏循证数据,SCIG 在慢性淋巴细胞白血病或多发性骨髓瘤患者中同样有效,并且在没有 B 细胞恢复证据的情况下停用 SCIG 会导致 IgG 下降和再次感染。最后,SAD 患者的治疗耐受性与 PAD 队列相当。总体而言,我们的数据表明,SCIG 已在 PAD 中得到认可,是 SAD 患者的一种有价值的选择,与导致抗体缺陷的疾病无关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/ed031c7907d9/pone.0247717.g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/86ebfff37a0a/pone.0247717.g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/68fa438df92b/pone.0247717.g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/703eea68cc9c/pone.0247717.g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/503d85bfa59e/pone.0247717.g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/ed031c7907d9/pone.0247717.g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/86ebfff37a0a/pone.0247717.g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/68fa438df92b/pone.0247717.g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/703eea68cc9c/pone.0247717.g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/503d85bfa59e/pone.0247717.g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8007/7932095/ed031c7907d9/pone.0247717.g005.jpg

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