{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T17:53:19Z","timestamp":1771005199262,"version":"3.50.1"},"reference-count":32,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":11149,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1976,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>25\u2010yr old female identical twins of Italian\u2010American origin concordant for sickle \u03b2\u2010thalassemia were studied to explain their clinical differences. One of them has been severely affected from childhood with one aplastic crisis, an earlier onset of vaso\u2010occlusive crises, and recent cardiac decompensation; the other twin shows no cardiac decompensation.<\/jats:p><jats:p>Similar are their degree of anemia, RBC indices, blood volumes, absence of splenic sequestration, depression of pO<jats:sub>2<\/jats:sub>, elevation of p50 and 2,3\u2010DPG, hemoglobin composition, and peripheral blood globin\u2010synthetic rates.<\/jats:p><jats:p>Regarding differences, the more severely affected has a shorter <jats:sup>51<\/jats:sup> Cr RBC life span, a greater menstrual blood loss, and is more overweight, whereas the less severely affected has functional asplenia by <jats:sup>99m<\/jats:sup>Tc scanning and a larger proportion of RBC with decreased cellular deformability.<\/jats:p><jats:p>We conclude that in sickle \u03b2\u2010thalassemia: (1) genotype alone does not determine the clinical course; (2) significant differences in clinical course can occur with almost identical hemoglobin composition and globin synthetic rates; (3) cellular deformability changes do not correlate exactly with clinical course; and (4) functional asplenia and leanness may be advantageous.<\/jats:p>","DOI":"10.1002\/ajh.2830010104","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T04:53:23Z","timestamp":1172033603000},"page":"23-33","source":"Crossref","is-referenced-by-count":9,"title":["Sickle \u03b2\u2010thalassemia: Identical twins differing in severity implicate nongenetic factors influencing course"],"prefix":"10.1002","volume":"1","author":[{"given":"Suresh K.","family":"Joishy","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul F.","family":"Griner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter T.","family":"Rowley","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","first-page":"146","volume-title":"The Thalassaemia Syndromes","author":"Weatherall D. 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