{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T20:40:12Z","timestamp":1790109612036,"version":"4.0.1"},"reference-count":25,"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":7618,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1985,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Irreversibly sickled cells (ISCs), considered by some to be of major pathophysiologic significance, have been reported to comprise between 5\u201350% of the total red cell population in patients with homozygous sickle cell anemia. Since the deformation of erythrocytes containing sickle hemoglobin is highly dependent on the concentration of hemoglobin in the deoxy conformation, any method established to enumerate the true ISC count requires the hemoglobin to be in the full oxy or liganded conformation. Because the oxygen dissociation curve for sickle erythrocytes is significantly shifted to the right, extremely high partial pressures of oxygen are required to approach full saturation. On the other hand, fully liganding the hemoglobin in the oxy conformation with carbon monoxide (CO) can be readily accomplished. We found that there is a significant reduction in the average number of sickled forms in the peripheral blood of sickle cell anemia patients after incubation in CO (to a value of 6.5 \u00b1 3.5%) when compared to conventional methods for ISC preparations. These results suggest that fully liganded erythrocytes should be used in quantitating ISCs in studies of the pathophysiology of this disease, especially since ISCs are likely to affect rheology differently from reversibly sickling cells.<\/jats:p>","DOI":"10.1002\/ajh.2830200104","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T10:22:02Z","timestamp":1172053322000},"page":"17-23","source":"Crossref","is-referenced-by-count":24,"title":["Irreversibly sickled erythrocytes in sickle cell anemia: A quantitative reappraisal"],"prefix":"10.1002","volume":"20","author":[{"given":"Griffin P.","family":"Rodgers","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constance Tom","family":"Noguchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan N.","family":"Schechter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","first-page":"117","article-title":"Relation of increased hypotonic fragility and of erythrostasis to the mechanism of hemolysis in certain anemia","volume":"55","author":"Ham TH","year":"1940","journal-title":"Trans Assoc Am Physicians"},{"key":"e_1_2_1_3_2","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1182\/blood.V47.4.621.621","article-title":"Delay time of gelation: A possible determinant of clinical severity in sickle cell disease","volume":"47","author":"Eaton WA","year":"1976","journal-title":"Blood"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197810052991405"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V64.3.733.733"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/44.1.1"},{"key":"e_1_2_1_7_2","doi-asserted-by":"crossref","first-page":"884","DOI":"10.1182\/blood.V33.6.884.884","article-title":"Reversible and irreversible sickling: A distinction by electron microscopy","volume":"33","author":"Bertles JF","year":"1969","journal-title":"Blood"},{"key":"e_1_2_1_8_2","first-page":"854","article-title":"In vivo study of the sickle cell phenomenon","volume":"56","author":"Jensen WN","year":"1960","journal-title":"J Lab Clin Med"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V41.5.653.653"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1969.tb01403.x"},{"key":"e_1_2_1_11_2","first-page":"235","volume-title":"Proceedings of the Symposium on Molecular and Cellular Aspects of Sickle Cell Disease","author":"Winslow RM","year":"1976"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V46.2.253.253"},{"key":"e_1_2_1_13_2","volume-title":"Statistical Methods","author":"Snedecor GW","year":"1980"},{"key":"e_1_2_1_14_2","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1182\/blood.V60.3.659.659","article-title":"A simple laboratory alternative to irreversibly sickled cell (ISC) counts","volume":"60","author":"Clark MR","year":"1982","journal-title":"Blood"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1939.02800080015004"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830020103"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/hon.2900040407"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198005013021803"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(63)91751-3"},{"key":"e_1_2_1_20_2","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1182\/blood.V42.6.835.835","article-title":"The role of red cell energy metabolism in the generation of irreversibly sickled cells in vitro","volume":"42","author":"Jensen M","year":"1973","journal-title":"Blood"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1042\/cs0480367"},{"key":"e_1_2_1_22_2","first-page":"146","article-title":"Effects of carbon dioxide and pH variations in vitro on blood respiratory functions, red blood cell volume, transmembrane pH gradients, and sickling in sickle cell anemia","volume":"104","author":"Ueda Y","year":"1984","journal-title":"J Lab Clin Med"},{"key":"e_1_2_1_23_2","unstructured":"RodgersGP SchechterAN NoguchiCT:Cell heterogeneity in sickle cell disease: Quantitation of erythrocyte density profile. 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