Acquired Hemophilia A and Autoimmunity. Study of Lymphocyte Populations and Myeloid-Derived Suppressor Cells
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 40
- 试验地点
- 2
- 主要终点
- Evaluate the evolution of lymphocyte populations and subpopulations, MDSCs and inflammatory cytokines in patients with hemophilia A acquired at diagnosis and during follow-up
研究概览
简要总结
Acquired hemophilia A is a rare condition of hemostasis secondary to the development of antibodies against factor VIII. This is a potentially serious pathology that can be life-threatening due to the major risk of bleeding caused by the sometimes drastic decrease in the level of circulating factor VIII.
This pathology occurs overwhelmingly in elderly subjects or, more rarely, in young women, during the postpartum period. It appears idiopathic in 50% of cases and associated, for the other cases, with underlying pathologies such as autoimmune pathologies (rheumatoid arthritis and bullous pemphigoid in particular) and neoplasias, or with a particular circumstance represented by the post -partum.
The association between this autoimmune pathology and its association with pathologies of the same type or with circumstances involving the immune system, suggests that common mechanisms could favor its emergence.
This study therefore proposes to study lymphocyte populations and subpopulations as well as Myeloid-Derived Suppressor Cells and the cytokine profile, which are abnormal in a large part of autoimmune pathologies.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Control
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •Patient population with acquired hemophilia A:
- •Adult patients with a diagnosis of acquired hemophilia A and with a social security system. Obtaining oral non-opposition from the patient to participate in the study following the submission of an information note relating to the project.
- •Obtaining signed and written informed consent for biocollection consent.
- •Population of Patients with constitutional hemophilia A:
- •Adult patients over 50 years of age with severe or moderate constitutional hemophilia A, with no history of autoimmune disease, and with a social security system.
- •Obtaining oral non-opposition from the patient to participate in the study following the submission of an information note relating to the project.
- •Obtaining signed and written informed consent for biocollection consent.
- •Population of control subjects:
- •Adult patients, over the age of 60, without a coagulation abnormality, with no history of autoimmune disease, and with a social security system.
- •Obtaining oral non-opposition from the patient to participate in the study following the submission of an information note relating to the project.
- •Obtaining signed and written informed consent for biocollection consent.
- •Patient population with inflammatory pathology:
- •Adult patients over the age of 50 with an inflammatory pathology likely to be associated with acquired hemophilia A, and with a social security system.
- •Obtaining oral non-opposition from the patient to participate in the study following the submission of an information note relating to the project.
- •Obtaining signed and written informed consent for biocollection consent.
排除标准
- •For the 4 groups:
- •Minor patient, under guardianship or curatorship.
- •Pregnant and lactating women.
- •Blood transfusion less than 7 days old.
- •Treatment with corticosteroids in the 7 days preceding inclusion or any other immunomodulatory or immunosuppressive treatment in the 4 weeks preceding inclusion.
结局指标
主要结局
Evaluate the evolution of lymphocyte populations and subpopulations, MDSCs and inflammatory cytokines in patients with hemophilia A acquired at diagnosis and during follow-up
时间窗: 2 years
Comparisons between diagnosis and during follow-up
次要结局
- Comparisons of baseline lymphocyte and cytokine data between patients with a favorable versus unfavorable final diagnosis.(2 years)
- To test the link between the severity of the disease at diagnosis and the cellular and cytokine parameters.(2 years)
- Comparison of lymphocyte populations and subpopulations, MDSCs and inflammatory cytokines between different groups.(2 years)