Clonal Hematopoiesis of Immunological Significance

Study Overview
=================
Brief Summary
-----------------
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.

Detailed Description
-----------------
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.

Official Title
-----------------
National Cohort of Patients With Dysimmune Manifestations With or Without Clonal Hematopoiesis

Conditions
-----------------
Immune System Diseases, Autoimmune Diseases, Inflammation, Autoinflammatory Diseases, Vexas Syndrome, Hematopoiesis Clonal, Clonal Hematopoiesis of Indeterminate Potential, Hematologic Diseases, Myelodysplastic-Myeloproliferative Diseases, Leukemia Myelomonocytic Chronic, Myelodysplastic Syndromes, Myeloproliferative Disorders, Lymphoproliferative Disorders, Lymphoma, Leukemia, Monoclonal Gammopathy of Undetermined Significance

Intervention / Treatment
-----------------
* Other: observational cohort study


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Age >=18 years old Confirmed dysimmune manifestations: clinical or biological abnormality or systemic disease Presence or absence of myeloid or lymphoid blood disease according to World Health Organization (WHO) classification Exclusion Criteria: Persons benefiting from special protection: adults under guardianship and curatorship; people hospitalized without their consent and not protected by law; persons deprived of liberty. Persons not affiliated to the social security system

Ages Eligible for Study
-----------------
Minimum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Dysimmune manifestations with or without clonal hematopoiesis<br>Dysimmune manifestations with or without clonal hematopoiesis | Other: observational cohort study<br>* observational cohort study<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Incidence of dysimmune manifestations associated with hematological disorders | Number of new cases | Baseline | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| VEXAS syndrome | Number of patients with VEXAS syndrome | 10 years | 
| Dysimmune manifestations other than VEXAS syndrome | Number of patients with dysimmune manifestations other than VEXAS syndrome | 10 years | 
| Myeloid hemopathy | Number of patients with myeloid hemopathy | 10 years | 
| Lymphoid hemopathy | Number of patients with lymphoid hemopathy | 10 years | 
| Clonal hematopoiesis of undeterminate potential | Number of patients with clonal hematopoiesis of undeterminate potential | 10 years | 
| Skin involvement | Number of patients with skin involvement | 10 years | 
| Musculoskeletal involvement | Number of patients with musculoskeletal involvement | 10 years | 
| Ocular involvement | Number of patients with ocular involvement | 10 years | 
| Vascular involvement | Number of patients with vascular involvement | 10 years | 
| Neurological involvement | Number of patients with neurological involvement | 10 years | 
| Digestive system involvement | Number of patients with digestive system involvement | 10 years | 
| Cardiac involvement | Number of patients with cardiac involvement | 10 years | 
| Pulmonary involvement | Number of patients with pulmonary involvement | 10 years | 
| Renal involvement | Number of patients with renal involvement | 10 years | 
| Therapeutic interventions received | Type and duration of therapeutic interventions received | 10 years | 
| Progression to acute myeloid leukemia | Number of patients who progressed to acute myeloid leukemia | 10 years | 
| Overall mortality | Overall mortality rate from all causes | 10 years | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Inflammation, Autoinflammatory Diseases, Vexas syndrome, Autoimmune Diseases, Clonal Hematopoiesis of Indeterminate Potential, Myelodysplastic-Myeloproliferative Diseases, Lymphoproliferative Disorders, Monoclonal Gammopathy of Undetermined Significance