Duraisingham Sai S, Buckland Matthew, Dempster John, Lorenzo Lorena, Grigoriadou Sofia, Longhurst Hilary J
Immunology Department, Barts Health NHS Trust, London, United Kingdom.
PLoS One. 2014 Jun 27;9(6):e100324. doi: 10.1371/journal.pone.0100324. eCollection 2014.
Secondary antibody deficiency can occur as a result of haematological malignancies or certain medications, but not much is known about the clinical and immunological features of this group of patients as a whole. Here we describe a cohort of 167 patients with primary or secondary antibody deficiencies on immunoglobulin (Ig)-replacement treatment. The demographics, causes of immunodeficiency, diagnostic delay, clinical and laboratory features, and infection frequency were analysed retrospectively. Chemotherapy for B cell lymphoma and the use of Rituximab, corticosteroids or immunosuppressive medications were the most common causes of secondary antibody deficiency in this cohort. There was no difference in diagnostic delay or bronchiectasis between primary and secondary antibody deficiency patients, and both groups experienced disorders associated with immune dysregulation. Secondary antibody deficiency patients had similar baseline levels of serum IgG, but higher IgM and IgA, and a higher frequency of switched memory B cells than primary antibody deficiency patients. Serious and non-serious infections before and after Ig-replacement were also compared in both groups. Although secondary antibody deficiency patients had more serious infections before initiation of Ig-replacement, treatment resulted in a significant reduction of serious and non-serious infections in both primary and secondary antibody deficiency patients. Patients with secondary antibody deficiency experience similar delays in diagnosis as primary antibody deficiency patients and can also benefit from immunoglobulin-replacement treatment.
继发性抗体缺陷可由血液系统恶性肿瘤或某些药物引起，但对于这组患者整体的临床和免疫学特征了解甚少。在此，我们描述了一组167例接受免疫球蛋白（Ig）替代治疗的原发性或继发性抗体缺陷患者。对其人口统计学、免疫缺陷病因、诊断延迟、临床和实验室特征以及感染频率进行了回顾性分析。B细胞淋巴瘤化疗以及使用利妥昔单抗、皮质类固醇或免疫抑制药物是该队列中继发性抗体缺陷的最常见原因。原发性和继发性抗体缺陷患者在诊断延迟或支气管扩张方面没有差异，两组均出现与免疫失调相关的病症。继发性抗体缺陷患者的血清IgG基线水平相似，但IgM和IgA水平较高，且转换记忆B细胞频率高于原发性抗体缺陷患者。两组还比较了Ig替代前后的严重和非严重感染情况。虽然继发性抗体缺陷患者在开始Ig替代前有更多严重感染，但治疗使原发性和继发性抗体缺陷患者的严重和非严重感染均显著减少。继发性抗体缺陷患者与原发性抗体缺陷患者的诊断延迟相似，也可从免疫球蛋白替代治疗中获益。
