PDF(Pubmed)
Abstract：
UNASSIGNED: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune disorder characterized by uncontrolled lymphocyte and macrophage activation and a subsequent cytokine storm. The timely initiation of immunosuppressive treatment is crucial for survival.
UNASSIGNED: Here, we harnessed Vγ9Vδ2 T cell degranulation to develop a novel functional assay for the diagnosis of HLH. We compared the novel assay with the conventional natural killer (NK) cell stimulation method in terms of efficiency, specificity, and reliability. Our analysis involved 213 samples from 182 individuals, including 23 samples from 12 patients with degranulation deficiency (10 individuals with UNC13D deficiency, 1 with STXBP2 deficiency, and 1 with RAB27A deficiency).
UNASSIGNED: While both tests exhibited 100% sensitivity, the Vγ9Vδ2 T cell degranulation assay showed a superior specificity of 86.2% (n=70) compared to the NK cell degranulation assay, which achieved 78.9% specificity (n=213). The Vγ9Vδ2 T cell degranulation assay offered simpler technical requirements and reduced labor intensity, leading to decreased susceptibility to errors with faster processing times.
UNASSIGNED: This efficiency stemmed from the sole requirement of dissolving (E)-4-hydroxy-3-methyl-but-2-enyl pyrophosphate (HMBPP) powder, contrasting with the intricate maintenance of K562 cells necessary for the NK cell degranulation assay. With its diminished susceptibility to errors, we anticipate that the assay will require fewer repetitions of analysis, rendering it particularly well-suited for testing infants.
UNASSIGNED: The Vγ9Vδ2 T cell degranulation assay is a user-friendly, efficient diagnostic tool for HLH. It offers greater specificity, reliability, and practicality than established methods. We believe that our present findings will facilitate the prompt, accurate diagnosis of HLH and thus enable rapid treatment and better patient outcomes.
UNASSIGNED: Here, we harnessed Vγ9Vδ2 T cell degranulation to develop a novel functional assay for the diagnosis of HLH. We compared the novel assay with the conventional natural killer (NK) cell stimulation method in terms of efficiency, specificity, and reliability. Our analysis involved 213 samples from 182 individuals, including 23 samples from 12 patients with degranulation deficiency (10 individuals with UNC13D deficiency, 1 with STXBP2 deficiency, and 1 with RAB27A deficiency).
UNASSIGNED: While both tests exhibited 100% sensitivity, the Vγ9Vδ2 T cell degranulation assay showed a superior specificity of 86.2% (n=70) compared to the NK cell degranulation assay, which achieved 78.9% specificity (n=213). The Vγ9Vδ2 T cell degranulation assay offered simpler technical requirements and reduced labor intensity, leading to decreased susceptibility to errors with faster processing times.
UNASSIGNED: This efficiency stemmed from the sole requirement of dissolving (E)-4-hydroxy-3-methyl-but-2-enyl pyrophosphate (HMBPP) powder, contrasting with the intricate maintenance of K562 cells necessary for the NK cell degranulation assay. With its diminished susceptibility to errors, we anticipate that the assay will require fewer repetitions of analysis, rendering it particularly well-suited for testing infants.
UNASSIGNED: The Vγ9Vδ2 T cell degranulation assay is a user-friendly, efficient diagnostic tool for HLH. It offers greater specificity, reliability, and practicality than established methods. We believe that our present findings will facilitate the prompt, accurate diagnosis of HLH and thus enable rapid treatment and better patient outcomes.
摘要：
■噬血细胞性淋巴组织细胞增生症(HLH)是一种威胁生命的免疫病症,其特征在于不受控制的淋巴细胞和巨噬细胞活化以及随后的细胞因子风暴。及时开始免疫抑制治疗对于生存至关重要。
■这里，我们利用Vγ9Vδ2T细胞脱颗粒来开发一种用于HLH诊断的新型功能检测方法。我们比较了新的测定与传统的自然杀伤（NK）细胞刺激方法在效率方面，特异性，和可靠性。我们的分析涉及182个人的213个样本，包括来自12名脱颗粒缺乏症患者的23个样本(10名UNC13D缺乏症患者，1患有STXBP2缺乏症，1患有RAB27A缺乏症)。
■虽然两个测试都表现出100%的灵敏度，Vγ9Vδ2T细胞脱颗粒试验显示出比NK细胞脱颗粒试验高86.2%(n=70)的特异性,特异性为78.9%(n=213)。Vγ9Vδ2T细胞脱颗粒测定提供了更简单的技术要求和降低的劳动强度，以更快的处理时间降低对错误的敏感性。
■这种效率源于溶解（E）-4-羟基-3-甲基-丁-2-烯基焦磷酸（HMBPP）粉末的唯一要求，与NK细胞脱颗粒测定所需的K562细胞的复杂维持相反。随着它对错误的敏感性降低，我们预计该测定将需要更少的重复分析，使其特别适合测试婴儿。
■Vγ9Vδ2T细胞脱颗粒测定法是一种用户友好的，高效的HLH诊断工具。它提供了更大的特异性，可靠性,和实用性比既定的方法。我们相信，我们目前的研究结果将有助于及时，准确诊断HLH，从而实现快速治疗和更好的患者预后。
■这里，我们利用Vγ9Vδ2T细胞脱颗粒来开发一种用于HLH诊断的新型功能检测方法。我们比较了新的测定与传统的自然杀伤（NK）细胞刺激方法在效率方面，特异性，和可靠性。我们的分析涉及182个人的213个样本，包括来自12名脱颗粒缺乏症患者的23个样本(10名UNC13D缺乏症患者，1患有STXBP2缺乏症，1患有RAB27A缺乏症)。
■虽然两个测试都表现出100%的灵敏度，Vγ9Vδ2T细胞脱颗粒试验显示出比NK细胞脱颗粒试验高86.2%(n=70)的特异性,特异性为78.9%(n=213)。Vγ9Vδ2T细胞脱颗粒测定提供了更简单的技术要求和降低的劳动强度，以更快的处理时间降低对错误的敏感性。
■这种效率源于溶解（E）-4-羟基-3-甲基-丁-2-烯基焦磷酸（HMBPP）粉末的唯一要求，与NK细胞脱颗粒测定所需的K562细胞的复杂维持相反。随着它对错误的敏感性降低，我们预计该测定将需要更少的重复分析，使其特别适合测试婴儿。
■Vγ9Vδ2T细胞脱颗粒测定法是一种用户友好的，高效的HLH诊断工具。它提供了更大的特异性，可靠性,和实用性比既定的方法。我们相信，我们目前的研究结果将有助于及时，准确诊断HLH，从而实现快速治疗和更好的患者预后。
