{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T03:34:18Z","timestamp":1780630458795,"version":"3.54.1"},"reference-count":38,"publisher":"Springer Science and Business Media LLC","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Exp Hematol Oncol"],"published-print":{"date-parts":[[2013,12]]},"DOI":"10.1186\/2162-3619-2-28","type":"journal-article","created":{"date-parts":[[2013,10,10]],"date-time":"2013-10-10T11:10:46Z","timestamp":1381403446000},"source":"Crossref","is-referenced-by-count":14,"title":["Utility of hydroxyurea in mast cell activation syndrome"],"prefix":"10.1186","volume":"2","author":[{"given":"Lawrence B","family":"Afrin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2013,10,9]]},"reference":[{"key":"66_CR1","doi-asserted-by":"publisher","first-page":"10","DOI":"10.1186\/1756-8722-4-10","volume":"4","author":"GJ Molderings","year":"2011","unstructured":"Molderings GJ, Brettner S, Homann J, Afrin LB: Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. J Hematol Oncol 2011, 4: 10. , accessed October 5, 2013 http:\/\/www.jhoonline.org\/content\/4\/1\/10 10.1186\/1756-8722-4-10","journal-title":"J Hematol Oncol"},{"key":"66_CR2","doi-asserted-by":"publisher","first-page":"158","DOI":"10.1097\/MOH.0b013e32834521dd","volume":"18","author":"PT McGann","year":"2011","unstructured":"McGann PT, Ware RE: Hydroyxurea for sickle cell anemia: what have we learned and what questions remain? Curr Opin Hematol 2011, 18: 158\u2013165. 10.1097\/MOH.0b013e32834521dd","journal-title":"Curr Opin Hematol"},{"key":"66_CR3","doi-asserted-by":"publisher","first-page":"1038","DOI":"10.1001\/jama.1966.03100120106029","volume":"195","author":"BJ Kennedy","year":"1966","unstructured":"Kennedy BJ, Yarbro JW: Metabolic and therapeutic effects of hydroxyurea in chronic myeloid leukemia. J Am Med Assoc 1966, 195: 1038\u20131043. 10.1001\/jama.1966.03100120106029","journal-title":"J Am Med Assoc"},{"key":"66_CR4","doi-asserted-by":"publisher","first-page":"1571","DOI":"10.1056\/NEJM198512193132503","volume":"313","author":"R Veith","year":"1985","unstructured":"Veith R, Galanello R, Papayannopoulou T, Stamatoyannopoulos G: Stimulation of F-cell production in patients with sickle cell anaemia treated with cytarabine or hydroxyurea. N Engl J Med 1985, 313: 1571\u20131575. 10.1056\/NEJM198512193132503","journal-title":"N Engl J Med"},{"key":"66_CR5","doi-asserted-by":"publisher","first-page":"1317","DOI":"10.1056\/NEJM199505183322001","volume":"332","author":"S Charache","year":"1995","unstructured":"Charache S, Terrin M, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR: Effect of hydroxyurea on the frequency of painful crisis in sickle cell anemia. N Engl J Med 1995, 332: 1317\u20131322. 10.1056\/NEJM199505183322001","journal-title":"N Engl J Med"},{"key":"66_CR6","doi-asserted-by":"publisher","first-page":"801","DOI":"10.1126\/science.7973634","volume":"266","author":"F Lori","year":"1994","unstructured":"Lori F, Malykh A, Cara A, Sun D, Weinstein JN, Lisziewicz J, Gallo RC: Hydroxyurea as an inhibitor of human immunodeficiency virus-type 1 replication. Science 1994, 266: 801\u2013805. 10.1126\/science.7973634","journal-title":"Science"},{"key":"66_CR7","doi-asserted-by":"publisher","first-page":"740","DOI":"10.1002\/ajh.20925","volume":"82","author":"UM Reiss","year":"2007","unstructured":"Reiss UM, Bensimhon P, Zimmerman SA, Ware RE: Hydroyxurea therapy for management of secondary erythrocytosis in cyanotic congenital heart disease. Am J Hematol 2007, 82: 740\u2013743. 10.1002\/ajh.20925","journal-title":"Am J Hematol"},{"key":"66_CR8","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1016\/0006-291X(68)90266-0","volume":"33","author":"HL Elford","year":"1968","unstructured":"Elford HL: Effect of hydroxyurea on ribonucleotide reductase. Biochem Biophys Res Commun 1968, 33: 129\u2013135. 10.1016\/0006-291X(68)90266-0","journal-title":"Biochem Biophys Res Commun"},{"key":"66_CR9","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1016\/j.amjmed.2004.06.032","volume":"117","author":"F Passamonti","year":"2004","unstructured":"Passamonti F, Rumi E, Pungolino E, Malabarba L, Bertazzoni P, Valentini M, Orlandi E, Arcaini L, Brusamolino E, Pascutto C, Cazzola M, Morra E, Lazzarino M: Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia. Am J Med 2004, 117: 755\u2013761. 10.1016\/j.amjmed.2004.06.032","journal-title":"Am J Med"},{"key":"66_CR10","doi-asserted-by":"publisher","first-page":"1132","DOI":"10.1056\/NEJM199504273321704","volume":"332","author":"S Cortelazzo","year":"1995","unstructured":"Cortelazzo S, Finazzi G, Ruggeri M, Vestri O, Galli M, Rodeghiero F, Barbui T: Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med 1995, 332: 1132\u20131136. 10.1056\/NEJM199504273321704","journal-title":"N Engl J Med"},{"key":"66_CR11","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1056\/NEJMoa043800","volume":"353","author":"CN Harrison","year":"2005","unstructured":"Harrison CN, Campbell PJ, Buck G, Wheatley K, East CL, Bareford D, Wilkins BS, van der Walt JD, Reilly JT, Grigg AP, Revell P, Woodcock BE, Green AR: Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med 2005, 353: 33\u201345. 10.1056\/NEJMoa043800","journal-title":"N Engl J Med"},{"key":"66_CR12","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1002\/ajh.21699","volume":"85","author":"MH Steinberg","year":"2010","unstructured":"Steinberg MH, McCarthy WF, Castro O, Ballas SK, Armstrong FD, Smith W, Ataga K, Swerdlow P, Kutlar A, DeCastro L, Waclawiw MA: The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: a 17.5 year follow-up. Am J Hematol 2010, 85: 403\u2013408.","journal-title":"Am J Hematol"},{"key":"66_CR13","doi-asserted-by":"publisher","first-page":"2039","DOI":"10.1182\/blood-2003-07-2475","volume":"103","author":"SA Zimmerman","year":"2004","unstructured":"Zimmerman SA, Schultz WH, Davis JS, Pickens CV, Mortier NA, Howard TA, Ware RE: Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerated dose in children with sickle cell disease. Blood 2004, 103: 2039\u20132045. 10.1182\/blood-2003-07-2475","journal-title":"Blood"},{"key":"66_CR14","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1016\/j.mrgentox.2010.03.001","volume":"698","author":"JM Flanagan","year":"2010","unstructured":"Flanagan JM, Howard TA, Mortier N, Avlasevich SL, Smeltzer MP, Wu S, Dertinger SD, Ware RE: Assessment of genotoxicity associated with hydroxyurea therapy in children with sickle cell anemia. Mutat Res 2010, 698: 38\u201342. 10.1016\/j.mrgentox.2010.03.001","journal-title":"Mutat Res"},{"key":"66_CR15","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1111\/j.1365-2141.2011.08698.x","volume":"154","author":"PT McGann","year":"2011","unstructured":"McGann PT, Howard TA, Flanagan JM, Lahti JM, Ware RE: Chromosome damage and repair in children with sickle cell anemia and long-term hydroxyurea exposure. Br J Haematol 2011, 154: 134\u2013140. 10.1111\/j.1365-2141.2011.08698.x","journal-title":"Br J Haematol"},{"key":"66_CR16","volume-title":"Am Soc Pediatr Hematol Oncol Annual Meeting","author":"JS Hankins","year":"2010","unstructured":"Hankins JS, Aygun B, Wang W, Wynn L, Thornburg C, Schultz W, Kimble A, Smeltzer M, Mortier N, Ware R: From infancy to adolescence: over a decade of continuous hydroxyurea therapy in sickle cell anemia [abstract]. Am Soc Pediatr Hematol Oncol Annual Meeting 2010. abstract 68, http:\/\/www.connect2conferences.com\/aspho4\/ws_search\/index.php?task=detail%abstract_id=68"},{"key":"66_CR17","doi-asserted-by":"publisher","first-page":"790","DOI":"10.1002\/ajh.21561","volume":"84","author":"KH Lim","year":"2009","unstructured":"Lim KH, Pardanani A, Butterfield JH, Li CY, Tefferi A: Cytoreductive therapy in 108 adults with systemic mastocytosis: outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, Imatinib mesylate or 2-chlorodeoxyadenosine. Am J Hematol 2009, 84: 790\u2013794. 10.1002\/ajh.21561","journal-title":"Am J Hematol"},{"key":"66_CR18","doi-asserted-by":"publisher","first-page":"922","DOI":"10.1111\/j.1365-4632.2004.02249.x","volume":"43","author":"LK Martin","year":"2004","unstructured":"Martin LK, Romanelli P, Ahn YS, Kirsner RS: Telangiectasia macularis eruptiva perstans with an associated myeloproliferative disorder. Int J Dermatol 2004, 43: 922\u2013924. 10.1111\/j.1365-4632.2004.02249.x","journal-title":"Int J Dermatol"},{"key":"66_CR19","doi-asserted-by":"publisher","first-page":"813","DOI":"10.1111\/j.1398-9995.2012.02812.x","volume":"67","author":"I Alvarez-Twose","year":"2012","unstructured":"Alvarez-Twose I, Va\u00f1\u00f3-Galv\u00e1n S, S\u00e1nchez-Mu\u00f1oz L, Morgado JM, Matito A, Torrelo A, Ja\u00e9n P, Schwartz LB, Orfao A, Escribano L: Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis. Allergy 2012, 67: 813\u2013821. 10.1111\/j.1398-9995.2012.02812.x","journal-title":"Allergy"},{"key":"66_CR20","doi-asserted-by":"publisher","first-page":"301","DOI":"10.1136\/jcp.52.4.301","volume":"52","author":"RJ Kennedy","year":"1999","unstructured":"Kennedy RJ, Scoffield JL, Garstin WI: An unusual presentation of systemic mastocytosis. J Clin Pathol 1999, 52: 301\u2013302. 10.1136\/jcp.52.4.301","journal-title":"J Clin Pathol"},{"key":"66_CR21","doi-asserted-by":"publisher","first-page":"1677","DOI":"10.1016\/0016-5085(95)90658-4","volume":"109","author":"TH Baron","year":"1995","unstructured":"Baron TH, Koehler RE, Rodgers WH, Fallon MB, Ferguson SM: Mast cell cholangiopathy: another cause of sclerosing cholangitis. Gastroenterol 1995, 109: 1677\u20131681. 10.1016\/0016-5085(95)90658-4","journal-title":"Gastroenterol"},{"key":"66_CR22","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1007\/s00277-007-0430-3","volume":"87","author":"CG Valentini","year":"2008","unstructured":"Valentini CG, Rondoni M, Pogliani EM, Van Lint MT, Cattaneo C, Marbello L, Pulsoni A, Giona F, Martinelli G, Leone G, Pagano L: Mast cell leukemia: a report of ten cases. Ann Hematol 2008, 87: 505\u2013508. 10.1007\/s00277-007-0430-3","journal-title":"Ann Hematol"},{"key":"66_CR23","doi-asserted-by":"publisher","first-page":"1215","DOI":"10.1038\/ni.f.216","volume":"9","author":"J Kalesnikoff","year":"2008","unstructured":"Kalesnikoff J, Galli SJ: New developments in mast cell biology. Nat Immunol 2008, 9: 1215\u20131223. 10.1038\/ni.f.216","journal-title":"Nat Immunol"},{"key":"66_CR24","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1016\/j.jaci.2004.04.046","volume":"114","author":"C Akin","year":"2004","unstructured":"Akin C, Metcalfe DD: The biology of Kit in disease and the application of pharmacogenetics. J Allergy Clin Immunol 2004, 114: 13\u201319. 10.1016\/j.jaci.2004.04.046","journal-title":"J Allergy Clin Immunol"},{"issue":"Suppl 3","key":"66_CR25","doi-asserted-by":"publisher","first-page":"19S","DOI":"10.1111\/1523-1747.ep12468945","volume":"96","author":"LJ Roberts","year":"1991","unstructured":"Roberts LJ, Oates JA: Biochemical diagnosis of systemic mast cell disorders. J Invest Derm 1991,96(Suppl 3):19S-25S.","journal-title":"J Invest Derm"},{"key":"66_CR26","doi-asserted-by":"publisher","first-page":"158","DOI":"10.1159\/000096442","volume":"142","author":"K Sonneck","year":"2007","unstructured":"Sonneck K, Florian S, M\u00fcllauer L, Wimazal F, F\u00f6dinger M, Sperr WR, Valent P: Diagnostic and subdiagnostic accumulation of mast cells in the bone marrow of patients with anaphylaxis: monoclonal mast cell activation syndrome. Int Arch Allergy Immunol 2007, 142: 158\u2013164. 10.1159\/000096442","journal-title":"Int Arch Allergy Immunol"},{"key":"66_CR27","doi-asserted-by":"publisher","first-page":"2331","DOI":"10.1182\/blood-2006-06-028100","volume":"110","author":"C Akin","year":"2007","unstructured":"Akin C, Scott LM, Kocabas CN, Kushnir-Sukhov N, Brittain E, Noel P, Metcalfe DD: Demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with \u201cidiopathic\u201d anaphylaxis. Blood 2007, 110: 2331\u20132333. 10.1182\/blood-2006-06-028100","journal-title":"Blood"},{"key":"66_CR28","doi-asserted-by":"publisher","first-page":"1045","DOI":"10.1080\/00365520701245744","volume":"42","author":"GJ Molderings","year":"2007","unstructured":"Molderings GJ, Kolck UW, Scheurlen C, Br\u00fcss M, Homann J, Von K\u00fcgelgen I: Multiple novel alterations in Kit tyrosine kinase in patients with gastrointestinally pronounced systemic mast cell activation disorder. Scand J Gastroenterol 2007, 42: 1045\u20131053. 10.1080\/00365520701245744","journal-title":"Scand J Gastroenterol"},{"key":"66_CR29","doi-asserted-by":"publisher","first-page":"721","DOI":"10.1007\/s00251-010-0474-8","volume":"62","author":"GJ Molderings","year":"2010","unstructured":"Molderings GJ, Meis K, Kolck UW, Homann J, Frieling T: Comparative analysis of mutation of tyrosine kinase Kit in mast cells from patients with systemic mast cell activation syndrome and healthy subjects. Immunogenetics 2010, 62: 721\u2013727. 10.1007\/s00251-010-0474-8","journal-title":"Immunogenetics"},{"key":"66_CR30","doi-asserted-by":"publisher","first-page":"1841","DOI":"10.1038\/leu.2008.233","volume":"22","author":"R Kralovics","year":"2008","unstructured":"Kralovics R: Genetic complexity of myeloproliferative neoplasms. Leukemia 2008, 22: 1841\u20131848. 10.1038\/leu.2008.233","journal-title":"Leukemia"},{"key":"66_CR31","doi-asserted-by":"publisher","first-page":"2460","DOI":"10.1182\/blood-2013-04-496448","volume":"122","author":"J Schwaab","year":"2013","unstructured":"Schwaab J, Schnittger S, Sotlar K, Walz C, Fabarius A, Pfirrmann M, Kohlmann A, Grossmann V, Meggendorfer M, Horny HP, Valent P, Jawhar M, Teichmann M, Metzgeroth G, Erben P, Ernst T, Hochhaus A, Haferlach T, Hofmann WK, Cross NC, Reiter A: Comprehensive mutational profiling in advanced systemic mastocytosis. Blood 2013, 122: 2460\u20132466. 10.1182\/blood-2013-04-496448","journal-title":"Blood"},{"key":"66_CR32","doi-asserted-by":"publisher","first-page":"1099","DOI":"10.1016\/j.jaci.2010.08.035","volume":"126","author":"C Akin","year":"2010","unstructured":"Akin C, Valent P, Metcalfe D: Mast cell activation syndrome: proposed diagnostic criteria. J Allergy Clin Immunol 2010, 126: 1099\u20131104.e4. 10.1016\/j.jaci.2010.08.035","journal-title":"J Allergy Clin Immunol"},{"key":"66_CR33","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1111\/j.1365-2567.2012.03627.x","volume":"137","author":"B Haenisch","year":"2012","unstructured":"Haenisch B, N\u00f6then MM, Molderings GJ: Systemic mast cell activation disease: the role of molecular genetic alterations in pathogenesis, heritability and diagnostics. Immunol 2012, 137: 197\u2013205. 10.1111\/j.1365-2567.2012.03627.x","journal-title":"Immunol"},{"key":"66_CR34","doi-asserted-by":"publisher","first-page":"1037","DOI":"10.1084\/jem.20082179","volume":"206","author":"Y Nakamura","year":"2009","unstructured":"Nakamura Y, Kambe N, Saito M, Nishikomori R, Kim YG, Murakami M, N\u00fa\u00f1ez G, Matsue H: Mast cells mediate neutrophil recruitment and vascular leakage through the NLRP3 inflammasome in histamine-independent urticaria. J Exp Med 2009, 206: 1037\u20131046. 10.1084\/jem.20082179","journal-title":"J Exp Med"},{"key":"66_CR35","doi-asserted-by":"publisher","first-page":"1853","DOI":"10.1182\/blood-2013-04-498105","volume":"122","author":"L Vincent","year":"2013","unstructured":"Vincent L, Vang D, Nguyen J, Gupta M, Luk K, Ericson ME, Simone DA, Gupta K: Mast cell activation contributes to sickle cell pathobiology and pain in mice. Blood 2013, 122: 1853\u20131862. 10.1182\/blood-2013-04-498105","journal-title":"Blood"},{"key":"66_CR36","doi-asserted-by":"publisher","first-page":"939","DOI":"10.7326\/0003-4819-148-12-200806170-00221","volume":"148","author":"S Lanzkron","year":"2008","unstructured":"Lanzkron S, Strouse JJ, Wilson R, Beach MC, Haywood C, Park H, Witkop C, Bass EB, Segal JB: Systematic review: hydroxyurea for the treatment of adults with sickle cell disease. Ann Intern Med 2008, 148: 939\u2013955. 10.7326\/0003-4819-148-12-200806170-00221","journal-title":"Ann Intern Med"},{"key":"66_CR37","doi-asserted-by":"crossref","first-page":"1078","DOI":"10.1182\/blood.V89.3.1078","volume":"89","author":"MH Steinberg","year":"1997","unstructured":"Steinberg MH, Lu ZH, Barton FB, Terrin ML, Charache S, Dover GJ: Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea. Blood 1997, 89: 1078\u20131088.","journal-title":"Blood"},{"key":"66_CR38","doi-asserted-by":"publisher","first-page":"e76241","DOI":"10.1371\/journal.pone.0076241","volume":"8","author":"GJ Molderings","year":"2013","unstructured":"Molderings GJ, Haenisch B, Bogdanow M, Fimmers R, N\u00f6then MM: Familial occurrence of systemic mast cell activation disease. PLoS ONE 2013, 8: e76241. 10.1371\/journal.pone.0076241","journal-title":"PLoS ONE"}],"container-title":["Experimental Hematology &amp; Oncology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/2162-3619-2-28.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,18]],"date-time":"2024-05-18T15:28:43Z","timestamp":1716046123000},"score":1,"resource":{"primary":{"URL":"https:\/\/ehoonline.biomedcentral.com\/articles\/10.1186\/2162-3619-2-28"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,10,9]]},"references-count":38,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2013,12]]}},"alternative-id":["66"],"URL":"https:\/\/doi.org\/10.1186\/2162-3619-2-28","relation":{},"ISSN":["2162-3619"],"issn-type":[{"value":"2162-3619","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,10,9]]},"article-number":"28"}}