{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,18]],"date-time":"2025-11-18T01:35:31Z","timestamp":1763429731331,"version":"3.40.5"},"reference-count":20,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,5,4]],"date-time":"2021-05-04T00:00:00Z","timestamp":1620086400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["eJHaem"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Understanding the interplay of genetic factors with haemoglobin expression and pathological processes in sickle cell disease is important for pharmacological and gene\u2010therapeutic interventions. In our nascent study cohort of Nigerian patients, we found that three major disease\u2010modifying factors, HbF levels, \u03b1\u2010thalassaemia deletion and <jats:italic>BCL11A<\/jats:italic> genotype, had expected beneficial haematological effects. A key <jats:italic>BCL11A<\/jats:italic> variant, while improving HbF levels (5.7%\u20139.0%), also led to a small, but significant decrease in HbA<jats:sub>2<\/jats:sub>. We conclude that in general, interventions boosting HbF are likely to reduce HbA<jats:sub>2<\/jats:sub> in patients\u2019 erythroid cells and that such therapeutic strategies might benefit from a parallel stimulation of HbA<jats:sub>2<\/jats:sub> through independent mechanisms.<\/jats:p>","DOI":"10.1002\/jha2.186","type":"journal-article","created":{"date-parts":[[2021,5,4]],"date-time":"2021-05-04T09:39:41Z","timestamp":1620121181000},"page":"459-461","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Fetal\u2010haemoglobin enhancing genotype at <i>BCL11A<\/i> reduces HbA<sub>2<\/sub> levels in patients with sickle cell anaemia"],"prefix":"10.1002","volume":"2","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2097-469X","authenticated-orcid":false,"given":"Titilope A.","family":"Adeyemo","sequence":"first","affiliation":[{"name":"Department of Haematology and Blood Transfusion, College of Medicine University of Lagos  Idi\u2010Araba Lagos Nigeria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oyesola O.","family":"Ojewunmi","sequence":"additional","affiliation":[{"name":"Sickle Cell Foundation Nigeria  Idi\u2010Araba Lagos Nigeria"},{"name":"School of Cancer and Pharmaceutical Sciences King's College London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Idayat Ajoke","family":"Oyetunji","sequence":"additional","affiliation":[{"name":"Sickle Cell Foundation Nigeria  Idi\u2010Araba Lagos Nigeria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olufunto Olufela","family":"Kalejaiye","sequence":"additional","affiliation":[{"name":"Haematology\/Oncology Unit, Department of Medicine, College of Medicine University of Lagos  Idi\u2010Araba Lagos Nigeria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1590-9108","authenticated-orcid":false,"given":"Stephan","family":"Menzel","sequence":"additional","affiliation":[{"name":"School of Cancer and Pharmaceutical Sciences King's College London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,5,4]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.76.2.670"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.90.11.5039"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1159\/000203622"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12084"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23811"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1987.tb01343.x"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0111464"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40291-018-0370-8"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0197927"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1993.tb04639.x"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/clinchem\/42.7.1113"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2326-11-2"},{"key":"e_1_2_7_14_1","first-page":"279","volume-title":"Sickle 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