Clonal Hematopoiesis of Immunological Significance

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.

This is an International, multicentre, observational cohort study with retrospective and prospective components (ambispective).

The primary objective is to describe the incidence of immuno-inflammatory manifestations in patients with clonal hematopoiesis or a haematological disease.

The secondary objectives are as follows:

* To describe the clinical and biological presentation of immuno-inflammatory manifestations according to the type of underlying haematological disease or clonal hematopoiesis; * To describe the clinical and biological presentation of VEXAS syndrome and its association with other haematological diseases; * To study the relationship between giant cell arteritis and clonal hematopoiesis; * To specify clinical symptoms according to the genetic mutations identified; * To define the main genetic mutations associated with these manifestations; * To identify patients eligible for different therapeutic trials; * To assess the characteristics of associated haematological diseases; * To compare the effectiveness of immunomodulatory and antitumour treatments according to the type of immuno-inflammatory manifestation and type of underlying haematological disease or clonal hematopoiesis; * To study the profile of patients eligible for stem cell transplantation; * To study mortality in patients followed for an inflammatory disease with or without haematological disease/clonal hematopoiesis; * To explore the natural history of patients over a 10-year follow-up in order to better characterise long-term complications; * To build a multicentre reference database enabling cross-sectional and longitudinal analyses to guide future therapeutic strategies; * To establish correlations between clinical, biological and molecular characteristics in order to better stratify risk and adapt patient management.

• VEXAS syndrome [Срок оценки: 10 years]
• Dysimmune manifestations other than VEXAS syndrome [Срок оценки: 10 years]
• Myeloid hemopathy [Срок оценки: 10 years]
• Lymphoid hemopathy [Срок оценки: 10 years]
• Clonal hematopoiesis of undeterminate potential [Срок оценки: 10 years]
• Skin involvement [Срок оценки: 10 years]
• Musculoskeletal involvement [Срок оценки: 10 years]
• Ocular involvement [Срок оценки: 10 years]
• Vascular involvement [Срок оценки: 10 years]
• Neurological involvement [Срок оценки: 10 years]
• Digestive system involvement [Срок оценки: 10 years]
• Cardiac involvement [Срок оценки: 10 years]