Hemophilia A
Conditions
Brief summary
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.
Interventions
OTHERno intervention
no intervention
Sponsors
Nantes University Hospital
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
1. 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. 2. 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. 3. 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. 4. 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.
Exclusion criteria
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Evaluate the evolution of lymphocyte populations and subpopulations, MDSCs and inflammatory cytokines in patients with hemophilia A acquired at diagnosis and during follow-up | 2 years | Comparisons between diagnosis and during follow-up |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| To test the link between the severity of the disease at diagnosis and the cellular and cytokine parameters. | 2 years | Factor VIII level and inhibitor titration at diagnosis |
| Comparisons of baseline lymphocyte and cytokine data between patients with a favorable versus unfavorable final diagnosis. | 2 years | Cell populations and cytokine profile and half-life of the inhibitor under treatment, time before normalization of the Factor VIII / Willebrand factor ratio, total duration of treatment with corticosteroids, recurrence and mortality tested to define a favorable course of the disease or unfavorable. |
| Comparison of lymphocyte populations and subpopulations, MDSCs and inflammatory cytokines between different groups. | 2 years | Cell populations and cytokine profile of the different groups at diagnosis and at the end of follow-up. |
Countries
France
Contacts
Primary ContactMarc Fouassier
Outcome results
None listed