{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T04:46:24Z","timestamp":1761540384803},"reference-count":28,"publisher":"American Society of Hematology","issue":"12","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2001,6,15]]},"abstract":"<jats:p>Thrombosis is a life-threatening complication of hemolytic anemia in humans. Cardiac thrombi are present in all adult \u03b1-spectrin\u2013deficient (sph\/sph) mice with severe hereditary spherocytosis, providing a model for events preceding thrombosis. The current study evaluated (1) the timing of thrombosis initiation and (2) the effect of postnatal transplantation of normal cells on life span and thrombotic incidence in adult mice. Thrombi are detected histologically following necropsy in untreatedsph\/sph mice of various ages and are not observed until 6 weeks of age. Thrombotic incidence increases from 50% at 6 to 7 weeks of age to 100% at 9 weeks of age. As a potential therapy, nonablatedsph\/sph neonates were transfused with either genetically marked normal peripheral blood (PB), bone marrow (BM), or both and assessed for donor cells and thrombosis. A single transfusion of PB, with or without BM, significantly increases the percentage ofsph\/sph mice that survive to weaning (4 weeks of age). Replacement in all sph\/sph recipients is limited to red blood cells (RBCs). RBCs derived from donor PB are lost within 5 weeks. PB plus BM prolongs high-level donor PB cell production better than BM alone. Thrombotic incidence is significantly reduced in allsph\/sph mice treated with PB, BM, or both. Hence, the presence of normal blood cells in the peripheral circulation of neonatal and adult sph\/sph mice rescues the former and abrogates the development of thrombosis in the latter.<\/jats:p>","DOI":"10.1182\/blood.v97.12.3972","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T12:46:47Z","timestamp":1027774007000},"page":"3972-3975","update-policy":"http:\/\/dx.doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":10,"title":["Reduced incidence of thrombosis in mice with hereditary spherocytosis following neonatal treatment with normal hematopoietic cells"],"prefix":"10.1182","volume":"97","author":[{"given":"Nancy J.","family":"Wandersee","sequence":"first","affiliation":[{"name":"From the Jackson Laboratory, Bar Harbor, ME."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John C.","family":"Lee","sequence":"additional","affiliation":[{"name":"From the Jackson Laboratory, Bar Harbor, ME."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susan A.","family":"Deveau","sequence":"additional","affiliation":[{"name":"From the Jackson Laboratory, Bar Harbor, ME."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jane E.","family":"Barker","sequence":"additional","affiliation":[{"name":"From the Jackson Laboratory, Bar Harbor, ME."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"234","reference":[{"key":"2020021300074222000_B1","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1002\/(SICI)1096-8652(199809)59:1<46::AID-AJH9>3.0.CO;2-#","article-title":"Prothrombin mutant, factor V Leiden, and thermolabile variant of methylenetetrahydrofolate reductase among patients with sickle cell disease in Brazil.","volume":"59","author":"Andrade","year":"1998","journal-title":"Am J Hematol."},{"key":"2020021300074222000_B2","first-page":"1645","article-title":"The sickle syndromes.","volume-title":"Blood: Principles and Practice of Hematology.","author":"Platt","year":"1995"},{"key":"2020021300074222000_B3","first-page":"965","article-title":"The spectrum of brain MR abnomalities in sickle cell disease: a report from the Cooperative Study of Sickle Cell Disease.","volume":"17","author":"Moser","year":"1996","journal-title":"Am J Neuroradiol."},{"key":"2020021300074222000_B4","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1016\/S0002-9343(97)00129-0","article-title":"Homocysteine in sickle cell disease: relationship to stroke.","volume":"103","author":"Houston","year":"1997","journal-title":"Am J Med."},{"key":"2020021300074222000_B5","first-page":"288","article-title":"Cerebrovascular accidents in sickle cell disease: rates and risk factors.","volume":"91","author":"Ohene-Frempong","year":"1998","journal-title":"Blood."},{"key":"2020021300074222000_B6","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1159\/000040814","article-title":"Thromboembolic events in beta thalassemia major: an Italian multicenter study.","volume":"99","author":"Borgna-Pignatti","year":"1998","journal-title":"Acta Haematologica."},{"key":"2020021300074222000_B7","first-page":"536","article-title":"Hereditary spherocytosis, elliptocytosis and related disorders.","volume-title":"Williams Hematology.","author":"Palek","year":"1995"},{"key":"2020021300074222000_B8","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1007\/s002470050302","article-title":"Moyamoya disease in a patient with hereditary spherocytosis.","volume":"28","author":"Holz","year":"1998","journal-title":"Pediatr Radiol."},{"key":"2020021300074222000_B9","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1002\/(SICI)1096-8652(199801)57:1<82::AID-AJH15>3.0.CO;2-B","article-title":"Hereditary spherocytosis, thrombosis, and chronic pulmonary emboli: a case report and review of the literature.","volume":"57","author":"Hayag-Barin","year":"1998","journal-title":"Am J Hematol."},{"key":"2020021300074222000_B10","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1177\/000331979704800811","article-title":"Excessive arterial thrombus in spherocytosis: a case report.","volume":"48","author":"Nikol","year":"1997","journal-title":"Angiology."},{"key":"2020021300074222000_B11","doi-asserted-by":"crossref","first-page":"4610","DOI":"10.1182\/blood.V90.11.4610","article-title":"Thrombosis and secondary hemochromatosis play major roles in the pathogenesis of jaundiced and spherocytic mice, murine models for hereditary spherocytosis.","volume":"90","author":"Kaysser","year":"1997","journal-title":"Blood."},{"key":"2020021300074222000_B12","doi-asserted-by":"crossref","first-page":"4856","DOI":"10.1182\/blood.V92.12.4856","article-title":"Hematopoietic cells from alpha-spectrin deficient mice are sufficient to induce thrombotic events in hematopoietically ablated recipients.","volume":"92","author":"Wandersee","year":"1998","journal-title":"Blood."},{"key":"2020021300074222000_B13","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1016\/0092-8674(84)90408-2","article-title":"Spectrin deficient inherited hemolytic anemias in the mouse: characterization by spectrin synthesis and mRNA activity in reticulocytes.","volume":"37","author":"Bodine","year":"1984","journal-title":"Cell."},{"key":"2020021300074222000_B14","doi-asserted-by":"crossref","first-page":"10099","DOI":"10.1073\/pnas.91.21.10099","article-title":"The murine mutation jaundiced is caused by replacement of an arginine with a stop codon in the mRNA encoding the ninth repeat of \u03b2-spectrin.","volume":"91","author":"Bloom","year":"1994","journal-title":"Proc Natl Acad Sci U S A."},{"key":"2020021300074222000_B15","doi-asserted-by":"crossref","first-page":"8121","DOI":"10.1073\/pnas.85.21.8121","article-title":"Chromosomal location of three spectrin genes: relationship to the inherited hemolytic anemias of mouse and man.","volume":"85","author":"Birkenmeier","year":"1988","journal-title":"Proc Natl Acad Sci U S A."},{"key":"2020021300074222000_B16","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1006\/bcmd.2000.0277","article-title":"Erythroid phosphatidyl serine exposure is not predictive of thrombotic risk in mice with hemolytic anemia.","volume":"26","author":"Wandersee","year":"2000","journal-title":"Blood Cells Mol Dis."},{"key":"2020021300074222000_B17","doi-asserted-by":"crossref","first-page":"7332","DOI":"10.1073\/pnas.85.19.7332","article-title":"Red and white blood cell replacement during repopulation of W\/Wv mice with limiting dilutions of +\/+ donor marrow cells.","volume":"85","author":"Barker","year":"1988","journal-title":"Proc Natl Acad Sci U S A."},{"key":"2020021300074222000_B18","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1016\/S0301-472X(99)00028-4","article-title":"Multiple high cell dose injections of normal marrow into newborn jaundiced mice dramatically prolong life despite transient repopulation.","volume":"27","author":"Barker","year":"1999","journal-title":"Exp Hematol."},{"key":"2020021300074222000_B19","doi-asserted-by":"crossref","first-page":"2014","DOI":"10.1182\/blood.V73.7.2014.2014","article-title":"Marrow transplantation in the treatment of a murine heritable hemolytic anemia.","volume":"73","author":"Barker","year":"1989","journal-title":"Blood."},{"key":"2020021300074222000_B20","first-page":"328","article-title":"Percutaneous intravenous injection in neonatal mice.","volume":"49","author":"Sands","year":"1999","journal-title":"Lab Anim Sci."},{"key":"2020021300074222000_B21","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1038\/269517a0","article-title":"Ovarian teratomas in mice are derived from oocytes that have completed the first meiotic division.","volume":"269","author":"Eppig","year":"1977","journal-title":"Nature."},{"key":"2020021300074222000_B22","doi-asserted-by":"crossref","first-page":"2122","DOI":"10.1182\/blood.V80.8.2122.2122","article-title":"Fetal compensation of the hemolytic anemia in mice homozygous for the normoblastosis (nb) mutation.","volume":"80","author":"Peters","year":"1992","journal-title":"Blood."},{"key":"2020021300074222000_B23","doi-asserted-by":"crossref","first-page":"985","DOI":"10.1016\/S0301-472X(00)00491-4","article-title":"Amelioration of severe hereditary spherocytosis in non-ablated adult mice by marrow transplantation.","volume":"28","author":"Barker","year":"2000","journal-title":"Exp Hematol."},{"key":"2020021300074222000_B24","doi-asserted-by":"crossref","first-page":"2573","DOI":"10.1182\/blood.V87.6.2573.bloodjournal8762573","article-title":"Phospholipase A2 levels in acute chest syndrome of sickle cell disease.","volume":"87","author":"Styles","year":"1996","journal-title":"Blood."},{"key":"2020021300074222000_B25","doi-asserted-by":"crossref","first-page":"4879","DOI":"10.1182\/blood.V87.11.4879.bloodjournal87114879","article-title":"Increased adhesion of erythrocytes to components of the extracellular matrix: isolation and characterization of a red blood cell lipid that binds thrombospondin and laminin.","volume":"87","author":"Hillery","year":"1996","journal-title":"Blood."},{"key":"2020021300074222000_B26","first-page":"594a","article-title":"Increased erythrocyte adhesion in mice with hereditary spherocytosis and hereditary elliptocytosis [abstract].","volume":"96","author":"Wandersee","year":"2000","journal-title":"Blood."},{"key":"2020021300074222000_B27","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1097\/00062752-199903000-00004","article-title":"Role of red cells in thrombosis.","volume":"6","author":"Andrews","year":"1999","journal-title":"Curr Opin Hematol."},{"key":"2020021300074222000_B28","doi-asserted-by":"crossref","first-page":"1584","DOI":"10.1056\/NEJM199711273372203","article-title":"Circulating activated endothelial cells in sickle cell anemia.","volume":"337","author":"Solovey","year":"1997","journal-title":"N Engl J Med."}],"container-title":["Blood"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/ashpublications.org\/blood\/article-pdf\/97\/12\/3972\/1675492\/h8120103972.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/ashpublications.org\/blood\/article-pdf\/97\/12\/3972\/1675492\/h8120103972.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,2,13]],"date-time":"2020-02-13T05:07:57Z","timestamp":1581570477000},"score":1,"resource":{"primary":{"URL":"https:\/\/ashpublications.org\/blood\/article\/97\/12\/3972\/107277\/Reduced-incidence-of-thrombosis-in-mice-with"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001,6,15]]},"references-count":28,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2001,6,15]]}},"URL":"https:\/\/doi.org\/10.1182\/blood.v97.12.3972","relation":{},"ISSN":["1528-0020","0006-4971"],"issn-type":[{"value":"1528-0020","type":"electronic"},{"value":"0006-4971","type":"print"}],"subject":[],"published":{"date-parts":[[2001,6,15]]}}}