{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T16:00:59Z","timestamp":1792425659535,"version":"4.5.0"},"reference-count":20,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2013,2,25]],"date-time":"2013-02-25T00:00:00Z","timestamp":1361750400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2013,3]]},"abstract":"<jats:p>Some patients with systemic mastocytosis have concurrent hematological neoplasms, designated in the World Health Organization (<jats:styled-content style=\"fixed-case\">WHO<\/jats:styled-content>) classification as systemic mastocytosis with associated clonal hematological non\u2010mast cell lineage disease (<jats:styled-content style=\"fixed-case\">SM\u2010AHNMD<\/jats:styled-content>). In this study, we analyzed 29 patients with <jats:styled-content style=\"fixed-case\">SM\u2010AHNMD<\/jats:styled-content> and compared them to 40 patients with pure <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>. The <jats:styled-content style=\"fixed-case\">AHNMDs<\/jats:styled-content> were classified as chronic myelomonocytic leukemia (<jats:styled-content style=\"fixed-case\">CMML<\/jats:styled-content>) (<jats:italic>n<\/jats:italic> = 10), myelodysplastic syndrome (<jats:styled-content style=\"fixed-case\">MDS<\/jats:styled-content>) (<jats:italic>n<\/jats:italic> = 7), myeloproliferative neoplasms (<jats:italic>n<\/jats:italic> = 4), B\u2010cell lymphoma\/leukemia\/plasma cell neoplasms (<jats:italic>n<\/jats:italic> = 7), and acute myeloid leukemia (<jats:italic>n<\/jats:italic> = 1). Patients with <jats:styled-content style=\"fixed-case\">SM\u2010AHNMD<\/jats:styled-content> were older, more frequently had constitutional symptoms and hematological abnormalities, less often had skin lesions, and had an inferior overall survival compared with pure <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> patients (48 months vs. not\u2010reached, <jats:italic>P<\/jats:italic> &lt; 0.001). Karyotypic abnormalities were detected in 9\/28 (32%) patients with <jats:styled-content style=\"fixed-case\">SM\u2010AHNMD<\/jats:styled-content> but not in pure <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> patients (<jats:italic>P<\/jats:italic> &lt; 0.001). Combined imaging\/ fluorescence\u2010in\u2010situ hybridization performed in four <jats:styled-content style=\"fixed-case\">SM\u2010AHNMD<\/jats:styled-content> cases revealed shared abnormal signals in mast cells and myeloid cells in two patients with <jats:styled-content style=\"fixed-case\">SM\u2010CMML<\/jats:styled-content> and one patient with <jats:styled-content style=\"fixed-case\">SM\u2010MDS<\/jats:styled-content>, but not in the mast cells of a case <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>\u2010associated with chronic lymphocytic leukemia with <jats:styled-content style=\"fixed-case\"><jats:italic>ATM<\/jats:italic><\/jats:styled-content>\u2010deletion. Quantitative mutation analysis showed higher levels of mutant <jats:styled-content style=\"fixed-case\"><jats:italic>KIT<\/jats:italic><\/jats:styled-content> <jats:styled-content style=\"fixed-case\">D816V<\/jats:styled-content> in <jats:styled-content style=\"fixed-case\">SM\u2010CMML<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">SM\u2010MDS<\/jats:styled-content> than in pure <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content> (<jats:italic>P<\/jats:italic> &lt; 0.001). Our data indicate that the <jats:styled-content style=\"fixed-case\">SM\u2010AHNMD<\/jats:styled-content> category in the <jats:styled-content style=\"fixed-case\">WHO<\/jats:styled-content> classification is heterogeneous, including clonally related and unrelated forms of <jats:styled-content style=\"fixed-case\">AHNMD<\/jats:styled-content>. The presentation, treatment, and outcome of patients with <jats:styled-content style=\"fixed-case\">SM\u2010AHNMD<\/jats:styled-content> is often dictated by the type of <jats:styled-content style=\"fixed-case\">AHNMD<\/jats:styled-content>. Am. J. Hematol. 88:219\u2013224, 2013. \u00a9 2012 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/ajh.23380","type":"journal-article","created":{"date-parts":[[2012,12,19]],"date-time":"2012-12-19T10:04:20Z","timestamp":1355911460000},"page":"219-224","source":"Crossref","is-referenced-by-count":85,"title":["Systemic mastocytosis with associated clonal hematological non\u2010mast cell lineage disease: Clinical significance and comparison of chomosomal abnormalities in <scp>SM<\/scp> and <scp>AHNMD<\/scp> components"],"prefix":"10.1002","volume":"88","author":[{"given":"Sa A.","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Hematopathology The University of Texas M.D. Anderson Cancer Center Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lloyd","family":"Hutchinson","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Massachusetts Medical Center Worcester Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guilin","family":"Tang","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas M.D. Anderson Cancer Center Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Su S.","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas M.D. Anderson Cancer Center Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia M.","family":"Miron","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Massachusetts Medical Center Worcester Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yang O.","family":"Huh","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas M.D. Anderson Cancer Center Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dan M.","family":"Jones","sequence":"additional","affiliation":[{"name":"Nichols Institute Chantilly Virginia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Bueso\u2010Ramos","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas M.D. Anderson Cancer Center Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Srdan","family":"Verstovsek","sequence":"additional","affiliation":[{"name":"Department of Leukemia University of Texas M.D. Anderson Cancer Center Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L. Jeffrey","family":"Medeiros","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas M.D. Anderson Cancer Center Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto N.","family":"Miranda","sequence":"additional","affiliation":[{"name":"Department of Hematopathology The University of Texas M.D. Anderson Cancer Center Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,2,25]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2003.04575.x"},{"key":"e_1_2_5_3_1","volume-title":"Mastocytosis (Mast Cell Disease)","author":"Horny H\u2010PMD BJ","year":"2008"},{"key":"e_1_2_5_4_1","first-page":"54","volume-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues","author":"Horny DDM","year":"2008"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-02-205237"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-05-220145"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2008.141"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.2003.014860"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V94.7.2333.419k30_2333_2342"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0065-2776(05)89005-4"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.2353\/jmoldx.2006.060022"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.2677"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-04-015545"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2404680"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.leukres.2007.04.008"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.leukres.2006.03.006"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.2003.012864"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.2353\/jmoldx.2008.070061"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.exphem.2007.08.016"},{"key":"e_1_2_5_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cancergencyto.2008.01.014"},{"key":"e_1_2_5_21_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2010.34.1610"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.23380","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.23380","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,16]],"date-time":"2023-10-16T09:25:14Z","timestamp":1697448314000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.23380"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,2,25]]},"references-count":20,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2013,3]]}},"alternative-id":["10.1002\/ajh.23380"],"URL":"https:\/\/doi.org\/10.1002\/ajh.23380","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,2,25]]}}}