Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency and has a broad spectrum of clinical manifestations. Among non-infectious complications, an increased incidence of malignancies may have a special relevance for survival, but little is known about treatment efficacy on malignant complications. This was a monocenter retrospective study on CVID patients, designed to provide preliminary data for the investigation of the possible link between therapeutic delay and tumor incidence. A total of 67 CVID subjects were included. The median diagnostic delay was 7.5 years (range: 0–63 years), and the median therapeutic delay was 8.5 years (range: 0–67 years). Malignancies were diagnosed in 18 (27%) patients. Eight out of 18 (44%) patients with a malignancy had lymphoma. Patients who developed a malignancy showed a longer therapeutic delay in comparison to patients with no malignancy, although no statistical significance was achieved (11 years vs 8 years, respectively, p = 0.424). We observed a lower frequency of malignancy in CVID patients with reduced therapeutic delay compared with patients with therapeutic delay ≥ 10 years. With a therapeutic delay of > 1 year, 74% had no tumor, and 25% had a tumor; with a therapeutic delay of > 10 years, 65% had no tumor and 35% had a malignancy. Among patients who had no malignancy, 64% had a therapeutic delay of < 10 years, and 36% had a therapeutic delay of ≥ 10 years. Among patients with malignancy, 47% of subjects had a therapeutic delay < 10 years, and 53% a therapeutic delay ≥ 10 years. The observation of clinical characteristics of our patients with CVID may suggest that an early institution of IgG replacement therapy could be of benefit for the prevention of malignant complications. Name of the registry: Comitato Etico Regionale delle Marche. Trial registration number: 1505. Date of registration: 27/10/2016, Retrospectively registered URL of trial registry record: http://www.ospedaliriuniti.marche.it/portale/archivio13_cerm-ancona_0_446_1.html. The trial was not registered before the first participant was enrolled

中文翻译：

---

根据治疗延迟的常见可变免疫缺陷患者的恶性肿瘤发病率：一项意大利回顾性单中心队列研究。

常见变异型免疫缺陷 (CVID) 是最常见的有症状的原发性免疫缺陷，具有广泛的临床表现。在非感染性并发症中，恶性肿瘤发病率的增加可能与生存具有特殊的相关性，但对恶性并发症的治疗效果知之甚少。这是一项针对 CVID 患者的单中心回顾性研究，旨在为研究治疗延迟与肿瘤发病率之间的可能联系提供初步数据。共包括 67 名 CVID 受试者。中位诊断延迟为 7.5 年（范围：0-63 年），中位治疗延迟为 8.5 年（范围：0-67 年）。18 名 (27%) 患者被诊断为恶性肿瘤。18 名 (44%) 恶性肿瘤患者中有 8 名患有淋巴瘤。与未发生恶性肿瘤的患者相比，发生恶性肿瘤的患者显示出更长的治疗延迟，尽管没有达到统计学意义（分别为 11 年和 8 年，p = 0.424）。我们观察到与治疗延迟 ≥ 10 年的患者相比，治疗延迟减少的 CVID 患者的恶性肿瘤发生率较低。治疗延迟 > 1 年，74% 没有肿瘤，25% 有肿瘤；治疗延迟 > 10 年，65% 没有肿瘤，35% 有恶性肿瘤。在没有恶性肿瘤的患者中，64% 的治疗延迟<10 年，36% 的治疗延迟≥10 年。在恶性肿瘤患者中，47% 的受试者治疗延迟<10 年，53% 的治疗延迟≥10 年。对我们的 CVID 患者临床特征的观察可能表明，早期进行 IgG 替代治疗可能有利于预防恶性并发症。注册名称：Comitato Etico Regionale delle Marche。试用注册号：1505。注册日期：27/10/2016，试用注册记录追溯注册网址：http://www.ospedaliriuniti.marche.it/portale/archivio13_cerm-ancona_0_446_1.html。在第一位参与者注册之前，该试验未注册 http://www.ospedaliriuniti.marche.it/portale/archivio13_cerm-ancona_0_446_1.html。在第一位参与者注册之前，该试验未注册 http://www.ospedaliriuniti.marche.it/portale/archivio13_cerm-ancona_0_446_1.html。在第一位参与者注册之前，该试验未注册