诱导耐受后，针对 IgA 过敏反应患者的长期治疗和正常血液成分输注。

Long-Term Treatment and Transfusion of Normal Blood Components Following Tolerance Induction in Patients with Anti-IgA Anaphylactic Reactions.

机构信息

Transfusion Medicine, Charité University Medicine - Campus Virchow Klinikum, and ZTB, Zentrum für Transfusionsmedizin und Zelluläre Therapie, Berlin, Germany.

出版信息

Transfus Med Hemother. 2014 Oct;41(5):381-7. doi: 10.1159/000366240. Epub 2014 Sep 15.

DOI:10.1159/000366240

PMID:25538541

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4264496/

Abstract

BACKGROUND

In general, patients with significant anti-Ig-A do not tolerate intravenous (i.v.) administration of normal blood products. Here, we present our experiences in the induction of immune tolerance (IIT) and long-term treatment in a series of such patients affected in such a way. The question whether blood components from IgA-deficient donors are required will be discussed.

METHODS

Ten adult patients (4 females and 6 males; age ranging from 36 to 75 years) with anti-IgA were included in this study. All patients required long-term treatment with blood components. One patient had IgA deficiency and paroxysmal nocturnal hemoglobinuria (PNH), and all other patients had common variable immunodeficiency (CVID). The particle gel immunoassay was used for the detection of anti-IgA. Immune tolerance to IgA was induced by controlled subcutaneous (s.c.) and/or i.v. infusions of IgG preparations.

RESULTS

Prior to IIT, anti-IgA was detectable in plasma samples of all patients and significantly diminished or abolished by controlled s.c. and/or i.v. infusions of IgG. Multiple transfusions with normal blood components could be repeatedly performed with the patient suffering from PNH without any complications. As long as i.v. IgG (IVIgG) infusions were consequently administered as individually required (intervals 2-8 weeks), none of the patients developed reactions during observation (up to 10 years). However, interruption of treatment and re-exposure to IVIgG resulted in adverse reactions.

CONCLUSION

Patients with significant anti-IgA can be safely desensitized and tolerate long-term IgG substitutions independent of the IgA concentration of the used blood component.

摘要

背景

一般来说，有显著抗 IgA 的患者不能耐受静脉（iv）输注正常血制品。在此，我们介绍了我们在一系列此类患者中诱导免疫耐受（IIT）和长期治疗的经验。是否需要来自 IgA 缺乏供体的血液成分的问题将被讨论。

方法

本研究纳入了 10 名成年患者（4 名女性和 6 名男性；年龄 36 至 75 岁），他们均有抗 IgA。所有患者均需要长期输注血液成分。1 名患者患有 IgA 缺乏症和阵发性夜间血红蛋白尿（PNH），其余所有患者均患有普通可变免疫缺陷（CVID）。使用粒子凝胶免疫分析法检测抗 IgA。通过控制皮下（s.c.）和/或静脉（i.v.）输注 IgG 制剂来诱导 IgA 免疫耐受。

结果

在 IIT 之前，所有患者的血浆样本中均可检测到抗 IgA，通过控制 s.c.和/或 i.v.输注 IgG 后，其显著减少或消除。患有 PNH 的患者可多次输注正常血制品而无任何并发症。只要根据需要（2-8 周间隔）连续输注静脉内 IgG（IVIgG），在观察期间（长达 10 年），没有患者发生反应。然而，中断治疗并重新暴露于 IVIgG 会导致不良反应。