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Here, we describe three children with severe SCA who have co\u2010inherited variants in genes for membrane proteins (<jats:italic>SPTA1<\/jats:italic> and <jats:italic>EPB1<\/jats:italic>) and <jats:italic>PIEZO1<\/jats:italic>. These cases suggest that variants in RBC membrane proteins may contribute to SCA severity and phenotypic variation. Co\u2010Inheritance of such variants could, in theory, affect outcomes of emerging SCA therapies, including genetic modification treatments.<\/jats:p>","DOI":"10.1002\/pbc.31937","type":"journal-article","created":{"date-parts":[[2025,8,5]],"date-time":"2025-08-05T05:09:39Z","timestamp":1754370579000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Severe Hemolytic Phenotype in Children With Sickle Cell Anemia and Co\u2010Inherited Red Blood Cell Variants"],"prefix":"10.1002","volume":"72","author":[{"given":"Meghana","family":"Srinivas","sequence":"first","affiliation":[{"name":"Department of Pediatrics East Tennessee State University School of Medicine  Johnson City Tennessee USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sue","family":"Jaspersen","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology Department of Pediatrics Washington University in St. Louis School of Medicine  St. Louis Missouri USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David B.","family":"Wilson","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology Department of Pediatrics Washington University in St. Louis School of Medicine  St. Louis Missouri USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8933-0046","authenticated-orcid":false,"given":"Monica L.","family":"Hulbert","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology Department of Pediatrics Washington University in St. Louis School of Medicine  St. Louis Missouri USA"},{"name":"Dana\u2010Farber\/Boston Children\u2019s Cancer and Blood Disorders Center Harvard Medical School Boston Massachusetts USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,8,4]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2006.07.001"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamanetworkopen.2023.37484"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-04-216861"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-03-143891"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1155\/2019\/3179173"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41586-020-2308-7"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.87.18.7339"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/940616"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.20049"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2018.02.047"},{"issue":"2","key":"e_1_2_8_12_1","first-page":"43","article-title":"[Hereditary elliptocytosis in West Africa: Frequency and repartition of spectrin variants]","volume":"306","author":"Lecomte M. 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