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Immuno\u2010haematological features, as cytopenias, hypogammaglobulinemia, hypereosinophilia, lymphoproliferation and immunodeficiency, have been described in association of several MSAID. The objective of this review was to describe these particular MSAID. MSAID must be suspected in front of immuno\u2010haematological features associated with non\u2010infectious recurrent fever, chronic systemic inflammation, inflammatory cutaneous manifestations, arthritis or inflammatory bowel disease. Genes and cellular mechanisms involved are various but some of them are of special interest. Defects in actine regulation pathway are notably associated with cytopenia and immune deficiency. Because of their frequency, ADA2 deficiency and Vacuoles, E1\u2010Enzyme, X\u2010linked, auto\u2010inflammatory, Somatic (VEXAS) syndrome deserve to be noticed. ADA2 deficiency results in polyarteritis nodosa\u2010like presentation with a wide panel of manifestations including cytopenia(s), lymphoproliferation and immune deficiency. Neutrophilic dermatosis or chondritis associated with macrocytic anaemia or myelodysplasia should lead to screen for VEXAS. Of note, most of MSAID are associated with inflammatory anaemia. We proposed here a clinical and pragmatic approach of MSAID associated with immuno\u2010haematological features.<\/jats:p>","DOI":"10.1111\/imm.13579","type":"journal-article","created":{"date-parts":[[2022,9,24]],"date-time":"2022-09-24T08:38:37Z","timestamp":1664008717000},"page":"1-17","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Break down the barriers of auto\u2010inflammation: How to deal with a monogenic auto\u2010inflammatory disease and immuno\u2010haematological features in 2022?"],"prefix":"10.1111","volume":"168","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7644-2903","authenticated-orcid":false,"given":"H\u00e9l\u00e8ne","family":"Vergneault","sequence":"first","affiliation":[{"name":"Internal Medicine Department APHP, Tenon Hospital, National Reference Center for Autoinflammatory Diseases and Inflammatory Amyloidosis (CEREMAIA), Sorbonne University  Paris 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