{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T15:05:32Z","timestamp":1761059132177},"reference-count":23,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2010,11,24]],"date-time":"2010-11-24T00:00:00Z","timestamp":1290556800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"NIH"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2011,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Treatment\u2010associated fetal hemoglobin (HbF) expression patterns in children with sickle cell disease (SCD) have not been fully described. The objective of this study was to compare HbF expression profiles (HbF and F\u2010cells) in the peripheral blood of pediatric SCD patients receiving hydroxyurea (HU), chronic transfusions (Tx) or no chronic therapy (Ctrl).<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>Peripheral blood samples were collected from SCD patients between 1 month and 21 years of age and immunostained with anti\u2010HbF and anti\u2010HbA antibodies. Erythrocytes containing HbF (F\u2010cells) were enumerated with this dual staining method. HbF was measured using chromatography (HPLC).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Blood from 44 Ctrl patients \u22644 years of age was compared with that from older children (50 Ctrl, 17 HU, 17 Tx). Among the older children, the percentage of both HbF and F\u2010cells in the Tx group was significantly decreased compared to the control (HbF 5.4\u2009\u00b1\u20094.2% vs. 11.0\u2009\u00b1\u20097.2%, <jats:italic>P<\/jats:italic>\u2009=\u20090.003; F\u2010cells 30.2\u2009\u00b1\u200916.3% vs. 43.8\u2009\u00b1\u200920.4%, <jats:italic>P<\/jats:italic>\u2009=\u20090.0071). While the distribution of F\u2010cells was significantly increased in the HU group (56.3\u2009\u00b1\u200917.1% vs. 43.8\u2009\u00b1\u200920.4%, <jats:italic>P<\/jats:italic>\u2009=\u20090.016), the increase in HbF was less robust (14.7\u2009\u00b1\u20096.4% vs. 11.0\u2009\u00b1\u20097.2%, <jats:italic>P<\/jats:italic>\u2009=\u20090.051). Positive correlations of HbF and F\u2010cell distributions were noted in all groups (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.0001 for all groups). In serial samples from individual patients, relatively static patterns of HbF and F\u2010cell distribution were noted.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Pediatric SCD patients possess distinct patterns of HbF switching and silencing in peripheral blood erythrocytes. Thereafter, erythrocyte HbF expression level and distribution are maintained with both patient\u2010 and treatment\u2010specific patterns that may be useful for predicting the need or response to HbF\u2010modulating therapy. Pediatr Blood Cancer. 2010;56:103\u2013109. \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.22643","type":"journal-article","created":{"date-parts":[[2010,11,24]],"date-time":"2010-11-24T14:56:51Z","timestamp":1290610611000},"page":"103-109","source":"Crossref","is-referenced-by-count":14,"title":["Expression patterns of fetal hemoglobin in sickle cell erythrocytes are both patient\u2010 and treatment\u2010specific during childhood"],"prefix":"10.1002","volume":"56","author":[{"given":"Emily Riehm","family":"Meier","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Colleen","family":"Byrnes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maxine","family":"Weissman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pierre","family":"Noel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naomi L.C.","family":"Luban","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffery L.","family":"Miller","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2010,11,24]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V84.9.3182.3182"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1994.02170080026005"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(85)80125-6"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199904013401307"},{"key":"e_1_2_6_6_2","first-page":"213","article-title":"Stroke in sickle cell disease: Demographic, clinical and therapeutic considerations","volume":"28","author":"Ohene\u2010Frempong K","year":"1991","journal-title":"Semin Hematol"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-09-008318"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199406093302303"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI2484"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(81)80529-X"},{"key":"e_1_2_6_11_2","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1182\/blood.V63.4.921.921","article-title":"Is there a threshold of fetal hemoglobin that ameliorates morbidity in sickle cell anemia?","volume":"63","author":"Powars DR","year":"1984","journal-title":"Blood"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198801143180207"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-04-015859"},{"key":"e_1_2_6_14_2","first-page":"197","article-title":"The cooperative study of sickle cell disease: Review of study design and objectives","volume":"4","author":"Gaston M","year":"1982","journal-title":"Am J Pediatr Hematol Oncol"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199505183322001"},{"key":"e_1_2_6_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(199701)54:1<40::AID-AJH6>3.0.CO;2-4"},{"key":"e_1_2_6_17_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2008-0441"},{"key":"e_1_2_6_18_2","first-page":"2442","volume-title":"Hematology: Basic principles and practice","author":"Hoffman R","year":"2008"},{"key":"e_1_2_6_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.exphem.2004.11.007"},{"key":"e_1_2_6_20_2","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-199909000-00013"},{"key":"e_1_2_6_21_2","doi-asserted-by":"publisher","DOI":"10.1007\/3-540-27310-7_8"},{"key":"e_1_2_6_22_2","doi-asserted-by":"publisher","DOI":"10.1038\/313320a0"},{"key":"e_1_2_6_23_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1994.tb55707.x"},{"key":"e_1_2_6_24_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2008.07501.x"}],"container-title":["Pediatric Blood &amp; 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