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In erythrocytes, PIEZO1 adapts the intracellular ionic content and cell hydration status to the mechanical constraints induced by the environment. Until recently, the pathophysiology of hereditary xerocytosis was mainly believed to be based on the \u201cPIEZO1\u2010Gardos channel axis\u201d in erythrocytes, according to which PIEZO1\u2010activating mutations induce a calcium influx that secondarily activates the Gardos channel, leading to potassium and water efflux and subsequently to red blood cell dehydration. However, recent studies have demonstrated additional roles for PIEZO1 during early erythropoiesis and reticulocyte maturation, as well as roles in other tissues and cells such as lymphatic vessels, hepatocytes, macrophages and platelets that may affect the pathophysiology of the disease. These findings, presented and discussed in this review, broaden our understanding of hereditary xerocytosis beyond that of primarily being a red blood cell disease and identify potential therapeutic targets.\n                  <\/jats:p>","DOI":"10.1002\/ajh.26192","type":"journal-article","created":{"date-parts":[[2021,4,14]],"date-time":"2021-04-14T00:24:18Z","timestamp":1618359858000},"page":"1017-1026","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":57,"title":["Recent advances in the pathophysiology of\n                    <scp>PIEZO1<\/scp>\n                    \u2010related hereditary xerocytosis"],"prefix":"10.1002","volume":"96","author":[{"given":"Nicolas","family":"Jankovsky","sequence":"first","affiliation":[{"name":"UR4666 HEMATIM Universit\u00e9 Picardie Jules Verne  Amiens France"},{"name":"Service d'H\u00e9matologie Clinique, CHU Amiens\u2010Picardie  Amiens 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