{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T01:06:47Z","timestamp":1790384807847,"version":"4.1.0"},"reference-count":46,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2016,11,23]],"date-time":"2016-11-23T00:00:00Z","timestamp":1479859200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"NIHR BTRU and the NHSBT (see Acknowledgment). National Institute for Health Research Blood and Transplant Unit (NIHR BTRU), in Red Blood Cell Products Unit, of University of Bristol, & NHSBT"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cytometry Part B Clinical"],"published-print":{"date-parts":[[2018,3]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Bone marrow examination has been the confirmatory test for congenital dyserythropoietic anemia type II (CDAII). Occasional spherocytes on peripheral blood smear can confound the diagnosis. Since a screening test is still unavailable, we explored the feasibility of using flow cytometry as a preliminary screening method.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Thirteen monoclonal antibodies with specificities for eight erythrocyte membrane proteins were used in FACS analysis to probe the cellular features of red cells from CDAII, normal adults, hereditary spherocytosis (HS), and cord red cells. Confocal microscopy was performed on normal and CDAII to determine the overall distribution of CD44 and CD47. Their expression levels on cultured erythroblasts were also analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The densely stained band 3 as seen in CDAII in gel electrophoresis was also obtained for Dantu phenotype. Likewise analysis of CDAII cases (<jats:italic>n<\/jats:italic>\u2009=\u200926) using the eosin\u20105\u2032maleimide (EMA) binding test found 57% of patients giving results either positive or in the grey area for HS. Enhanced fluorescence of CD44 was detected in 96% of the CDAII patients, and anti\u2010CD47 binding was also elevated to a lesser degree. Although RNA expressions of <jats:italic>CD44<\/jats:italic> and <jats:italic>CD47<\/jats:italic> in the cultured erythroblasts of normal controls and CDAII were similar, confocal microscopy revealed more CDAII red cells giving elevated fluorescence than normal red cells.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>A distinction between CDAII and HS can be made using the EMA Binding test and anti\u2010CD44 binding. Confirmation of CDAII can subsequently be made based on clinical presentation together with either bone marrow examination or DNA sequencing of <jats:italic>SEC23B<\/jats:italic>. \u00a9 2016 International Clinical Cytometry Society<\/jats:p><\/jats:sec>","DOI":"10.1002\/cyto.b.21488","type":"journal-article","created":{"date-parts":[[2016,11,23]],"date-time":"2016-11-23T09:09:00Z","timestamp":1479892140000},"page":"312-326","source":"Crossref","is-referenced-by-count":11,"title":["CD44 as a Potential Screening Marker for Preliminary Differentiation Between Congenital Dyserythropoietic Anemia Type II and Hereditary Spherocytosis"],"prefix":"10.1002","volume":"94","author":[{"given":"B. 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J.","family":"Wo\u017aniak","sequence":"additional","affiliation":[{"name":"Bristol Institute for Transfusion Sciences, NHS Blood and Transplant Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Ranjha","sequence":"additional","affiliation":[{"name":"Bristol Institute for Transfusion Sciences, NHS Blood and Transplant Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Seeney","sequence":"additional","affiliation":[{"name":"Statistics and Clinical Studies NHS Blood and Transplant Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Bomford","sequence":"additional","affiliation":[{"name":"Institute of Liver Studies, King's College Hospital NHS Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Mehta","sequence":"additional","affiliation":[{"name":"Department of Haematology Bristol Royal Infirmary, North Bristol NHS Trust  UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Guest","sequence":"additional","affiliation":[{"name":"International Blood Group Reference Laboratory NHS Blood and Transplant Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Mushens","sequence":"additional","affiliation":[{"name":"International Blood Group Reference Laboratory NHS Blood and Transplant Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.\u2010J.","family":"King","sequence":"additional","affiliation":[{"name":"International Blood Group Reference Laboratory NHS Blood and Transplant Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,11,23]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.2010.01440.x"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23800"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1159\/000322948"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.12527"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijlh.12335"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.14271"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2005.05757.x"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/jclinpath-2016-203611"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00319637"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)60894-4"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2008.12.005"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V87.10.4433.bloodjournal87104433"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.405"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.21077"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2009.014985"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21866"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-02-0613"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2257.2001.00354.x"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2011.052845"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/cyto.b.21187"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2003.04730.x"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1751-553X.2010.01270.x"},{"key":"e_1_2_6_24_1","doi-asserted-by":"crossref","first-page":"4009","DOI":"10.4049\/jimmunol.134.6.4009","article-title":"Flow cytometric characterisation of normal and variant cells with monoclonal antibodies specific for glycophorin A","volume":"134","author":"Langlois RG","year":"1985","journal-title":"J Immunol"},{"key":"e_1_2_6_25_1","first-page":"197","article-title":"New monoclonal antibodies in CD44 and CD58: their use to quantify CD44 and CD58 on normal human erythrocytes and to compare the distribution of CD44 and CD58 in human tissues","volume":"74","author":"Anstee DJ","year":"1991","journal-title":"Immunology"},{"key":"e_1_2_6_26_1","unstructured":"SpringFA GardnerB JudsonPA AnsteeDJ.AS10.9 Epitope mapping of CD44 mAb. 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