{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T17:40:14Z","timestamp":1791222014240,"version":"4.1.0"},"reference-count":35,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2013,3,11]],"date-time":"2013-03-11T00:00:00Z","timestamp":1362960000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":904,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Lab Hematology"],"published-print":{"date-parts":[[2013,6]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>This overview describes two groups of nonimmune hereditary hemolytic anemias caused by defects in membrane proteins located in distinct layers of the red cell membrane. Hereditary spherocytosis (<jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content>), hereditary elliptocytosis (<jats:styled-content style=\"fixed-case\">HE<\/jats:styled-content>), and hereditary pyropoikilocytosis (<jats:styled-content style=\"fixed-case\">HPP<\/jats:styled-content>) represent disorders of the red cell cytoskeleton. Hereditary stomatocytoses represents disorders of cation permeability in the red cell membrane. The current laboratory screening tests for <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content> are the osmotic fragility test, acid glycerol lysis time test (<jats:styled-content style=\"fixed-case\">AGLT<\/jats:styled-content>), cryohemolysis test, and eosin\u20105\u2032\u2010maleimide (<jats:styled-content style=\"fixed-case\">EMA<\/jats:styled-content>)\u2010binding test. For atypical <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> SDS<\/jats:styled-content>\u2010polyacrylamide gel electrophoresis of erythrocyte membrane proteins is carried out to confirm the diagnosis. The diagnosis of <jats:styled-content style=\"fixed-case\">HE<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">HPP<\/jats:styled-content> is based on abnormal red cell morphology and the detection of protein 4.1R deficiency or spectrin variants using gel electrophoresis. None of screening tests can detect all <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content> cases. Some testing centers (a survey of 25 laboratories) use a combination of tests (e.g., <jats:styled-content style=\"fixed-case\">AGLT<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">EMA<\/jats:styled-content>). No specific screening test for hereditary stomatocytoses is available. The preliminary diagnosis is based on presenting a compensated hemolytic anemia, macrocytosis, and a temperature or time dependent pseudohyperkalemia in some patients. Both the <jats:styled-content style=\"fixed-case\">EMA<\/jats:styled-content>\u2010binding test and the osmotic fragility test may help in differential diagnosis of <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content> and hereditary stomatocytosis.<\/jats:p>","DOI":"10.1111\/ijlh.12070","type":"journal-article","created":{"date-parts":[[2013,3,13]],"date-time":"2013-03-13T02:42:47Z","timestamp":1363142567000},"page":"237-243","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":110,"title":["Hereditary red cell membrane disorders and laboratory diagnostic testing"],"prefix":"10.1111","volume":"35","author":[{"given":"M.\u2010J.","family":"King","sequence":"first","affiliation":[{"name":"Membrane Biochemistry NHS Blood and Transplant Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Zanella","sequence":"additional","affiliation":[{"name":"Hematology Unit Foundation IRCCS C\u00e1 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