{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,19]],"date-time":"2023-10-19T08:13:31Z","timestamp":1697703211217},"reference-count":20,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2009,3,3]],"date-time":"2009-03-03T00:00:00Z","timestamp":1236038400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2009,7,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Children with Down syndrome and acute megakaryoblastic leukemia (DS\u2010AMKL) have been shown to have increased sensitivity to cytarabine based chemotherapy. The excellent prognosis in patients with DS\u2010AMKL may be due to mutations in the <jats:italic>GATA1<\/jats:italic> gene leading to reduced expression of the enzyme cytidine deaminase. This leads to a decreased ability to convert cytarabine into its inactive metabolite, resulting in high intracellular concentration of this cytotoxic agent. We report two cases of DS\u2010AMKL with <jats:italic>GATA1<\/jats:italic> mutations who had poor outcome. These patients had high expression levels of cytidine deaminase mRNA transcripts. We speculate that other factors can affect overall outcome in patients with DS\u2010AMKL irrespective of the presence of <jats:italic>GATA1<\/jats:italic> mutations. Pediatr Blood Cancer 2009;53:108\u2013111. \u00a9 2009 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/pbc.21983","type":"journal-article","created":{"date-parts":[[2009,3,4]],"date-time":"2009-03-04T01:26:16Z","timestamp":1236129976000},"page":"108-111","source":"Crossref","is-referenced-by-count":1,"title":["<i>GATA1<\/i> mutations in patients with down syndrome and acute megakaryoblastic leukaemia do not always confer a good prognosis"],"prefix":"10.1002","volume":"53","author":[{"given":"Hany","family":"Ariffin","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jaime Castillo","family":"Garcia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Siti Sarah","family":"Daud","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kamariah","family":"Ibrahim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nik","family":"Aizah","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gek\u2010Bee","family":"Ong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lee\u2010Ai","family":"Chong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zulqarnain","family":"Mohamad","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2009,3,3]]},"reference":[{"key":"e_1_2_5_2_2","first-page":"608","article-title":"Distinctive demography, biology, and outcome of acute myeloid leukemia and myelodysplastic syndrome in children with Down syndrome: Children's Cancer Group Studies 2861 and 2891","volume":"91","author":"Lange BJ","year":"1998","journal-title":"Blood"},{"key":"e_1_2_5_3_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V80.9.2210.2210"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2403814"},{"key":"e_1_2_5_5_2","doi-asserted-by":"publisher","DOI":"10.3109\/08880018709141272"},{"key":"e_1_2_5_6_2","first-page":"1677","article-title":"Myelodysplasia and acute myelogenous leukemia in Down's syndrome. 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