{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T04:13:58Z","timestamp":1790050438442,"version":"4.0.1"},"reference-count":5,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2018,8,31]],"date-time":"2018-08-31T00:00:00Z","timestamp":1535673600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Severe iron overload is frequent in dehydrated hereditary stomatocytosis (DHSt) despite well\u2010compensated hemolysis and no or little transfusion requirement. We investigated 4 patients with proven <jats:styled-content style=\"fixed-case\">DHS<\/jats:styled-content>t, in whom the degree of hemolysis was closely related to iron status. Genetic modifiers increasing iron stores (<jats:styled-content style=\"fixed-case\">HFE<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">pC<\/jats:styled-content>ys282Tyr, <jats:styled-content style=\"fixed-case\">HAMP<\/jats:styled-content>:c\u2010153C&gt;T mutations) were accompanied with high liver iron concentrations and increased hemolysis, whereas therapeutic phlebotomies alleviated the hemolytic phenotype. There were no manifestations of hemolysis in one patient with low iron stores. Hemolysis reappeared when iron supplementation was given. The search for genetic or acquired modifiers of iron status and the modulation of iron stores may help in the management of these patients.<\/jats:p>","DOI":"10.1111\/ejh.13135","type":"journal-article","created":{"date-parts":[[2018,7,3]],"date-time":"2018-07-03T21:09:19Z","timestamp":1530652159000},"page":"566-569","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Inherited or acquired modifiers of iron status may dramatically affect the phenotype in dehydrated hereditary stomatocytosis"],"prefix":"10.1111","volume":"101","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1849-0186","authenticated-orcid":false,"given":"Corentin","family":"Orvain","sequence":"first","affiliation":[{"name":"Department of Haematology Biology Saint Eloi Hospital Montpellier University Hospital  Montpellier France"},{"name":"Department of Blood Diseases Angers University Hospital  Angers France"},{"name":"CRCINA, INSERM Universit\u00e9 de Nantes Universit\u00e9 d'Angers  Angers France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lydie","family":"Da Costa","sequence":"additional","affiliation":[{"name":"Department of Biological Haematology AP\u2010HP Robert Debr\u00e9 University Hospital  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Van Wijk","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry and Haematology Utrecht University Medical Center  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serge","family":"Pissard","sequence":"additional","affiliation":[{"name":"Department of Molecular Genetics AP\u2010HP Henri Mondor University Hospital  Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V\u00e9ronique","family":"Picard","sequence":"additional","affiliation":[{"name":"Laboratory of Haematology AP\u2010HP Bic\u00eatre University Hospital  Le Kremlin\u2010Bic\u00eatre France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lamisse","family":"Mansour\u2010Hendili","sequence":"additional","affiliation":[{"name":"Department of Haematology Biology Saint Eloi Hospital Montpellier University Hospital  Montpellier France"},{"name":"Department of Molecular Genetics AP\u2010HP Henri Mondor University Hospital  Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9verine","family":"Cunat","sequence":"additional","affiliation":[{"name":"Department of Haematology Biology Saint Eloi Hospital Montpellier University Hospital  Montpellier France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muriel","family":"Giansily\u2010Blaizot","sequence":"additional","affiliation":[{"name":"Department of Haematology Biology Saint Eloi Hospital Montpellier University Hospital  Montpellier 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