{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T14:57:57Z","timestamp":1791817077397,"version":"4.3.4"},"reference-count":27,"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":4696,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1993,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Homozygous \u03b1\u2010thalassemia [\u03b1\u2010thal\u20101], with loss of all four \u03b1\u2010globin genes, causes lethal hydrops fetalis. The most common mutation producing this syndrome is the Southeast Asian (\u2013<jats:sup><jats:sc>SEA<\/jats:sc><\/jats:sup>) double \u03b1\u2010globin gene deletion. Erythrocytes from adults heterozygous for the (\u2013<jats:sup><jats:sc>SEA<\/jats:sc><\/jats:sup>) deletion have minute amounts of embryonic \u03b6\u2010globin chains detectable by anti\u2010\u03b6\u2010globin monoclonal antibodies.<\/jats:p><jats:p>Among 225 peripheral blood samples tested by a simple anti\u2010\u03b6\u2010immunobinding tetra\u2010zolium dye test, 81 were positive and 144 were negative. The majority of subjects were of Filipino, Chinese, or Laotian ancestry. All 81 positive samples were confirmed by Bam HI digests and a \u03b6\u2010cDNA probe to have the (\u2013<jats:sup><jats:sc>SEA<\/jats:sc><\/jats:sup>) mutation. The (\u2013<jats:sup><jats:sc>SEA<\/jats:sc><\/jats:sup>) double \u03b1\u2010deletion was the only abnormality in 58. In the others, it was combined with \u03b1\u2010globin or \u03b2\u2010globin mutations, or coincidental iron deficiency. Four other samples from (\u2013<jats:sup><jats:sc>SEA<\/jats:sc><\/jats:sup>) heterozygotes were negative by this immunologic assay.<\/jats:p><jats:p>Anti\u2010\u03b6 negative samples included 78 deletions of the total \u03b1\u2010globin region, (\u2013<jats:sup>Tot<\/jats:sup>), 23 single \u03b1\u2010globin deletions, and a variety of \u03b2\u2010globin mutations; 16 normocytic samples with normal \u03b1\u2010genes were also negative. Ten anti\u2010\u03b6 positive and 25 anti\u2010\u03b6 negative samples had benign triplicated \u03b6\u2010globin genes.<\/jats:p><jats:p>In this population, the sensitivity of this test was 95%; and specificity for the (\u2013<jats:sup><jats:sc>SEA<\/jats:sc><\/jats:sup>) mutation was 100%. Anti\u2010\u03b6 immunobinding testing provides rapid, simple, and reliable screening for the (\u2013<jats:sup><jats:sc>SEA<\/jats:sc><\/jats:sup>) double \u03b1\u2010globin deletion, although it does not detect the (\u2013<jats:sup>Tot<\/jats:sup>) total \u03b1\u2010deletions. \u00a9 1993 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.2830440106","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T13:13:10Z","timestamp":1172063590000},"page":"22-28","source":"Crossref","is-referenced-by-count":6,"title":["Detection of the (\u2013<sup><scp>SEA<\/scp><\/sup>) double \u03b1\u2010globin gene deletion by a simple immunologic assay for embryonic \u03b6\u2010globin chains"],"prefix":"10.1002","volume":"44","author":[{"given":"James H.","family":"Ireland","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong\u2010Yuan","family":"Luo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David H. 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