剖析继发性免疫缺陷:识别 B 细胞淋巴组织增生性疾病中的原发性免疫缺陷。

IF 7.2 2区 医学 Q1 IMMUNOLOGY Journal of Clinical Immunology Pub Date : 2024-10-23 DOI:10.1007/s10875-024-01818-2
María Palacios-Ortega, Teresa Guerra-Galán, Adolfo Jiménez-Huete, José María García-Aznar, Marc Pérez-Guzmán, Maria Dolores Mansilla-Ruiz, Ángela Villegas Mendiola, Cristina Pérez López, Elsa Mayol Hornero, Alejandro Peixoto Rodriguez, Ascensión Peña Cortijo, Marta Polo Zarzuela, Marta Mateo Morales, Eduardo Anguita Mandly, Maria Cruz Cárdenas, Alejandra Carrero, Carlos Jiménez García, Estefanía Bolaños, Belén Íñigo, Fiorella Medina, Eduardo de la Fuente, Juliana Ochoa-Grullón, Blanca García-Solís, Yolanda García-Carmona, Miguel Fernández-Arquero, Celina Benavente-Cuesta, Rebeca Pérez de Diego, Nicholas Rider, Silvia Sánchez-Ramón
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引用次数: 0

摘要

区分原发性(PID)和继发性(SID)免疫缺陷,尤其是与血液学 B 细胞淋巴细胞增生性疾病(B-CLPD)相关的免疫缺陷,是一项重大的临床挑战。我们的目的是分析和定义与 B-CLPD 相关的 SID 患者的临床和实验室变量,找出与晚发性 PID 的重叠之处,从而有可能提高诊断的精确性和预后评估。我们研究了151名患有B-CLPD的SID患者的37个临床/实验室变量。根据ESID的主要PID标准,如果患者在B-CLPD之前有反复严重感染和/或低丙种球蛋白血症,则被归类为 "疑似PID组"。双变量关联分析显示,"疑似 PID 组 "和 "SID 组 "在 37 个分析变量中的 10 个变量上存在显著的统计学差异,其中 "疑似 PID 组 "在基线时的儿童复发性严重感染、B-CLPD 家族史、血清游离轻链(sFLC)显著较低、免疫球蛋白浓度、白细胞总数和开关记忆 B 细胞计数较低。我们采用了 Rpart 机器学习算法来创建一个模型,以区分这两组患者。该模型建立了一个决策树,其中两个主要变量的相关性依次为:κ+λ之和和儿童期严重复发感染史,对PID预测的灵敏度为89.5%,特异性为100%,准确率为91.8%。鉴别重要的临床和免疫学变量有助于在 SID 患者中识别晚发 PID 这一艰巨任务,强调了全面免疫学评估的价值。疑似 PID "组和 SID 组之间的差异凸显了早期定制诊断和治疗策略的必要性,以便对患者进行个性化管理和随访。
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Dissecting Secondary Immunodeficiency: Identification of Primary Immunodeficiency within B-Cell Lymphoproliferative Disorders.

Distinguishing between primary (PID) and secondary (SID) immunodeficiencies, particularly in relation to hematological B-cell lymphoproliferative disorders (B-CLPD), poses a major clinical challenge. We aimed to analyze and define the clinical and laboratory variables in SID patients associated with B-CLPD, identifying overlaps with late-onset PIDs, which could potentially improve diagnostic precision and prognostic assessment. We studied 37 clinical/laboratory variables in 151 SID patients with B-CLPD. Patients were classified as "Suspected PID Group" when having recurrent-severe infections prior to the B-CLPD and/or hypogammaglobulinemia according to key ESID criteria for PID. Bivariate association analyses showed significant statistical differences between "Suspected PID"- and "SID"-groups in 10 out of 37 variables analyzed, with "Suspected PID" showing higher frequencies of childhood recurrent-severe infections, family history of B-CLPD, significantly lower serum Free Light Chain (sFLC), immunoglobulin concentrations, lower total leukocyte, and switch-memory B-cell counts at baseline. Rpart machine learning algorithm was performed to potentially create a model to differentiate both groups. The model developed a decision tree with two major variables in order of relevance: sum κ + λ and history of severe-recurrent infections in childhood, with high sensitivity 89.5%, specificity 100%, and accuracy 91.8% for PID prediction. Identifying significant clinical and immunological variables can aid in the difficult task of recognizing late-onset PIDs among SID patients, emphasizing the value of a comprehensive immunological evaluation. The differences between "Suspected PID" and SID groups, highlight the need of early, tailored diagnostic and treatment strategies for personalized patient management and follow up.

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来源期刊
CiteScore
12.20
自引率
9.90%
发文量
218
审稿时长
2 months
期刊介绍: The Journal of Clinical Immunology publishes impactful papers in the realm of human immunology, delving into the diagnosis, pathogenesis, prognosis, or treatment of human diseases. The journal places particular emphasis on primary immunodeficiencies and related diseases, encompassing inborn errors of immunity in a broad sense, their underlying genotypes, and diverse phenotypes. These phenotypes include infection, malignancy, allergy, auto-inflammation, and autoimmunity. We welcome a broad spectrum of studies in this domain, spanning genetic discovery, clinical description, immunologic assessment, diagnostic approaches, prognosis evaluation, and treatment interventions. Case reports are considered if they are genuinely original and accompanied by a concise review of the relevant medical literature, illustrating how the novel case study advances the field. The instructions to authors provide detailed guidance on the four categories of papers accepted by the journal.
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