Clonal Hematopoiesis of Immunological Significance

Not yet recruiting

Conditions: Hematopoiesis Clonal
Hematologic Diseases
Leukemia Myelomonocytic Chronic
Myelodysplastic Syndromes
Autoinflammatory Diseases
Clonal Hematopoiesis of Indeterminate Potential
Monoclonal Gammopathy of Undetermined Significance
Autoimmune Diseases
Myeloproliferative Disorders
Immune System Diseases

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.

Recruitment & Eligibility

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

Arm && Interventions

Group	Intervention	Description
Dysimmune manifestations with or without clonal hematopoiesis	observational cohort study	Dysimmune manifestations with or without clonal hematopoiesis

Primary Outcome Measures

Name	Time	Method
Incidence of dysimmune manifestations associated with hematological disorders	Baseline	Number of new cases

Secondary Outcome Measures

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