Ambispective, national, multicenter observational cohort study aimed at characterizing the satellite dysimmune manifestations of clonal hematopoiesis, including Vexas (Vacuoles, E1 enzyme, X-linked, Autoinflammatory and Somatic) syndrome.
The clinical spectrum of dysimmune manifestations associated with blood diseases is wide. The pathophysiology of these manifestations is not well understood and their management is poorly codified. This observational cohort aims to list the different clinical pictures, the therapeutic management and the prognosis of patients according to the type of dysimmune manifestations and the type of hemopathy. We wish to have an inventory of the demographic, genetic, clinical and evolutionary data of patients with an inflammatory manifestation associated or not with a myeloid or lymphoid hemopathy. This will make it possible to establish quantitative data on the morbidity and mortality of these rare diseases and to propose therapeutic trials for the most serious patients.
Study Type
OBSERVATIONAL
Enrollment
1,000
observational cohort study
Incidence of dysimmune manifestations associated with hematological disorders
Number of new cases
Time frame: Baseline
VEXAS syndrome
Number of patients with VEXAS syndrome
Time frame: 10 years
Dysimmune manifestations other than VEXAS syndrome
Number of patients with dysimmune manifestations other than VEXAS syndrome
Time frame: 10 years
Myeloid hemopathy
Number of patients with myeloid hemopathy
Time frame: 10 years
Lymphoid hemopathy
Number of patients with lymphoid hemopathy
Time frame: 10 years
Clonal hematopoiesis of undeterminate potential
Number of patients with clonal hematopoiesis of undeterminate potential
Time frame: 10 years
Skin involvement
Number of patients with skin involvement
Time frame: 10 years
Musculoskeletal involvement
Number of patients with musculoskeletal involvement
Time frame: 10 years
Ocular involvement
Number of patients with ocular involvement
Time frame: 10 years
Vascular involvement
Number of patients with vascular involvement
Time frame: 10 years
Neurological involvement
Number of patients with neurological involvement
Time frame: 10 years
Digestive system involvement
Number of patients with digestive system involvement
Time frame: 10 years
Cardiac involvement
Number of patients with cardiac involvement
Time frame: 10 years
Pulmonary involvement
Number of patients with pulmonary involvement
Time frame: 10 years
Renal involvement
Number of patients with renal involvement
Time frame: 10 years
Therapeutic interventions received
Type and duration of therapeutic interventions received
Time frame: 10 years
Progression to acute myeloid leukemia
Number of patients who progressed to acute myeloid leukemia
Time frame: 10 years
Overall mortality
Overall mortality rate from all causes
Time frame: 10 years
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