Autoantibodies Against Type I Interferon in Patients Affected With Ph-negative Myeloproliferative Neoplasms (MPN-IFN Study)
试验速览
- 阶段
- 不适用
- 状态
- 进行中（未招募）
- 发起方
- 入组人数
- 219
- 试验地点
- 1
- 主要终点
- To evaluate for the prevalence of AAbs neutralizing IFN-I in a cohort of Ph-neg MPN patients.
研究概览
简要总结
The classic Ph-negative myeloproliferative neoplasms (MPN) are a group of clonal hematopoietic disorders caused by a dysregulated JAK/STAT signal transduction because of acquired somatic mutations of JAK2, CALR or MPL genes. Chronic inflammation may predispose to MPN development.
SARS-CoV-2 infection displays extreme interindividual clinical variability, ranging from asymptomatic infection to life-threatening coronavirus disease (COVID-19). Age is a major risk factor for severe disease. Male sex and medical comorbidities have minor impact. It was demonstrated that at least 3.5% of patients with life-threatening COVID-19 have monogenic inborn errors of TLR3- or TLR7-dependent type I interferons (IFN-I) immunity. It has also been described that at least 15% of patients with life-threatening COVID-19 have neutralizing autoantibodies (AAbs) against IFN-I, that precede SARS-CoV-2 infection.
As regard MPN, COVID-19 is associated with a mortality as high as 33%. Patients with MF had the highest mortality (48%), while patients with ET had the greatest risk of venous thromboembolism (16.7%).
In this prospective study, we want to search for AAbs against IFN-I in a cohort of MPN patients to evaluate their prevalence in the MPN population and to look for clinical correlations, including COVID-19 severity.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Only
- 时间视角
- Cross Sectional
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •A diagnosis of PV, ET, prePMF, overt PMF or MPN-U according to 2016 WHO criteria
- •Characterization of the MPN driver mutation performed at any moment before enrolment
- •A peripheral blood (PB) sample collected at the enrolment visit suitable for plasma extraction and functional evaluation of anti-cytokine auto-Abs
排除标准
- 未提供
结局指标
主要结局
To evaluate for the prevalence of AAbs neutralizing IFN-I in a cohort of Ph-neg MPN patients.
时间窗: 2 years
次要结局
- To compare the prevalence of AAbs neutralizing IFN-I in a cohort of Ph-neg MPN patients to that estimated in the general population.(2 years)
- To check for association between the presence/subtype/title of AAbs to IFN-I and both clinical and molecular features of MPN patients(2 years)
- To check for association between use of specific cytoreductive therapy and the presence of AAbs to IFN-I in MPN patients(2 years)
- To check for association between presence/subtype/title of AAbs to IFN-I and COVID-19 severity among MPN patients who get SARS-CoV-2 infection(2 years)
研究者
Elisa Rumi
MD
Fondazione IRCCS Policlinico San Matteo di Pavia