{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T18:21:39Z","timestamp":1793125299612,"version":"4.5.0"},"reference-count":32,"publisher":"American Society of Hematology","issue":"1","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2005,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Disorders of the erythrocyte membrane, including hereditary spherocytosis, hereditary elliptocytosis, hereditary pyropoikilocytosis, and hereditary stomatocytosis, comprise an important group of inherited hemolytic anemias. These syndromes are characterized by marked clinical and laboratory heterogeneity. Recent molecular studies have revealed that there is also significant genetic heterogeneity in these disorders. This is particularly true for the spherocytosis syndromes where each kindred has a private mutation in one of the spherocytosis genes.<\/jats:p><jats:p>Treatment with splenectomy is curative in most patients. Splenectomy via a laparoscopic approach has become the surgical method of choice. Growing recognition and understanding of the long-term risks and complications of splenectomy, including cardiovascular disease, thrombotic disorders, and pulmonary hypertension, and the emergence of penicillin-resistant pneumococci, a concern for infection in overwhelming postsplenectomy infection, have led to reevaluation of the role of splenectomy. Recent management guidelines acknowledge these important considerations when entertaining splenectomy and recommend detailed discussion between health care providers, patient, and family.<\/jats:p>","DOI":"10.1182\/asheducation-2005.1.13","type":"journal-article","created":{"date-parts":[[2005,11,23]],"date-time":"2005-11-23T01:29:34Z","timestamp":1132709374000},"page":"13-18","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":110,"title":["Red Cell Membrane Disorders"],"prefix":"10.1182","volume":"2005","author":[{"given":"Patrick G.","family":"Gallagher","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","reference":[{"key":"2019111704395235600_R1","unstructured":"Iolascon A, Perrotta S, Stewart GW. Red blood cell membrane defects. Rev Clin Exp Hematol. 2003;7:22\u201356."},{"key":"2019111704395235600_R2","doi-asserted-by":"crossref","unstructured":"Tse WT, Lux SE. Red blood cell membrane disorders. Br J Haematol. 1999;104:2\u201313.","DOI":"10.1111\/j.1365-2141.1999.01130.x"},{"key":"2019111704395235600_R3","doi-asserted-by":"crossref","unstructured":"Eber S, Lux SE. Hereditary spherocytosis\u2014defects in proteins that connect the membrane skeleton to the lipid bilayer. Semin Hematol. 2004;41:118\u2013141.","DOI":"10.1053\/j.seminhematol.2004.01.002"},{"key":"2019111704395235600_R4","unstructured":"Gallagher PG. Update on the clinical spectrum and genetics of red blood cell membrane disorders. Curr Hematol Rep. 2004;3:85\u201391."},{"key":"2019111704395235600_R5","doi-asserted-by":"crossref","unstructured":"Tamary H, Aviner S, Freud E, et al. High incidence of early cholelithiasis detected by ultrasonography in children and young adults with hereditary spherocytosis. J Pediatr Hematol Oncol. 2003;25:952\u2013954.","DOI":"10.1097\/00043426-200312000-00009"},{"key":"2019111704395235600_R6","doi-asserted-by":"crossref","unstructured":"del Giudice EM, Perrotta S, Nobili B, Specchia C, d\u2019Urzo G, Iolascon A. Coinheritance of Gilbert syndrome increases the risk for developing gallstones in patients with hereditary spherocytosis. Blood. 1999;94:2259\u20132262.","DOI":"10.1182\/blood.V94.7.2259.419k42_2259_2262"},{"key":"2019111704395235600_R7","doi-asserted-by":"crossref","unstructured":"King MJ, Smythe JS, Mushens R. Eosin-5-maleimide binding to band 3 and Rh-related proteins forms the basis of a screening test for hereditary spherocytosis. Br J Haematol. 2004;124:106\u2013113.","DOI":"10.1046\/j.1365-2141.2003.04730.x"},{"key":"2019111704395235600_R8","doi-asserted-by":"crossref","unstructured":"Reliene R, Mariani M, Zanella A, et al. Splenectomy prolongs in vivo survival of erythrocytes differently in spectrin\/ ankyrin- and band 3-deficient hereditary spherocytosis. Blood. 2002;100:2208\u20132215.","DOI":"10.1182\/blood.V100.6.2208.h81802002208_2208_2215"},{"key":"2019111704395235600_R9","doi-asserted-by":"crossref","unstructured":"Rescorla FJ, Engum SA, West KW, Tres Scherer LR, 3rd, Rouse TM, Grosfeld JL. Laparoscopic splenectomy has become the gold standard in children. Am Surg. 2002;68:297\u2013301; discussion 301\u2013292.","DOI":"10.1177\/000313480206800315"},{"key":"2019111704395235600_R10","doi-asserted-by":"crossref","unstructured":"Balague C, Targarona EM, Cerdan G, et al. Long-term outcome after laparoscopic splenectomy related to hematologic diagnosis. Surg Endosc. 2004;18:1283\u20131287.","DOI":"10.1007\/s00464-003-9092-y"},{"key":"2019111704395235600_R11","doi-asserted-by":"crossref","unstructured":"Bader-Meunier B, Gauthier F, Archambaud F, et al. Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis. Blood. 2001;97:399\u2013403.","DOI":"10.1182\/blood.V97.2.399"},{"key":"2019111704395235600_R12","doi-asserted-by":"crossref","unstructured":"Rice HE, Oldham KT, Hillery CA, Skinner MA, O\u2019Hara SM, Ware RE. Clinical and hematologic benefits of partial splenectomy for congenital hemolytic anemias in children. Ann Surg. 2003;237:281\u2013288.","DOI":"10.1097\/01.SLA.0000048453.61168.8F"},{"key":"2019111704395235600_R13","doi-asserted-by":"crossref","unstructured":"de Buys Roessingh AS, de Lagausie P, Rohrlich P, Berrebi D, Aigrain Y. Follow-up of partial splenectomy in children with hereditary spherocytosis. 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Br J Haematol. 2004;126:455\u2013474.","DOI":"10.1111\/j.1365-2141.2004.05052.x"},{"key":"2019111704395235600_R17","doi-asserted-by":"crossref","unstructured":"Gallagher PG. Hereditary elliptocytosis: spectrin and protein 4.1R. Semin Hematol. 2004;41:142\u2013164.","DOI":"10.1053\/j.seminhematol.2004.01.003"},{"key":"2019111704395235600_R18","doi-asserted-by":"crossref","unstructured":"Delaunay J. Molecular basis of red cell membrane disorders. Acta Haematol. 2002;108:210\u2013218.","DOI":"10.1159\/000065657"},{"key":"2019111704395235600_R19","doi-asserted-by":"crossref","unstructured":"Zhang Z, Weed SA, Gallagher PG, Morrow JS. Dynamic molecular modeling of pathogenic mutations in the spectrin self-association domain. Blood. 2001;98:1645\u20131653.","DOI":"10.1182\/blood.V98.6.1645"},{"key":"2019111704395235600_R20","doi-asserted-by":"crossref","unstructured":"Delaunay J, Stewart G, Iolascon A. Hereditary dehydrated and overhydrated stomatocytosis: recent advances. 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