{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T04:59:49Z","timestamp":1787979589586,"version":"build-2784847793"},"reference-count":32,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2003,4,11]],"date-time":"2003-04-11T00:00:00Z","timestamp":1050019200000},"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":[[2003,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The majority of patients with systemic mastocytosis with associated clonal, hematological non\u2010mast cell lineage disease (SM\u2010AHNMD) have a myeloid stem cell malignancy including myelodysplastic syndromes (MDS), myelodysplastic\/myeloproliferative disorders, acute myeloid leukemia (AML), or chronic myeloproliferative disease. The clinicopathologic features of SM\u2010AHNMD have not been fully characterized. We describe seven cases of this entity: 3 with MDS, 3 with AML, and 1 with chronic myelomonocytic leukemia. In the majority of cases, SM was diagnosed concurrently with the myeloid malignancy and aberrant mast cell morphology was observed. The commonly described <jats:italic>c\u2010kit<\/jats:italic> enzymatic site mutation Asp816Val was detected only in 2 cases, while 3 patients carried the Asp816His mutation. Among the 3 cases with AML, 2 patients carried the translocation t(8;21). On the basis of our results and other reported cases, there appears to be a specific association between SM and AML with t(8;21). Concurrent occurrence of SM may define a subset of patients with de novo AML and other myeloid malignancies who have an adverse prognosis. As clinically effective tyrosine kinase inhibitors that inhibit enzymatic\u2010type <jats:italic>c\u2010kit<\/jats:italic> mutations are being developed, detection of mast cell proliferation associated with myeloid malignancy may have important therapeutic implications. Am. J. Hematol. 73:12\u201317, 2003. \u00a9 2003 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.10322","type":"journal-article","created":{"date-parts":[[2003,4,14]],"date-time":"2003-04-14T10:02:38Z","timestamp":1050314558000},"page":"12-17","source":"Crossref","is-referenced-by-count":66,"title":["Systemic mastocytosis with associated clonal hematological non\u2010mast\u2010cell lineage disease: Analysis of clinicopathologic features and activating <i>c\u2010kit<\/i> mutations"],"prefix":"10.1002","volume":"73","author":[{"given":"Vinod A.","family":"Pullarkat","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Bueso\u2010Ramos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymond","family":"Lai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven","family":"Kroft","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carla S.","family":"Wilson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheeja T.","family":"Pullarkat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiangdong","family":"Bu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maung","family":"Thein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell K.","family":"Brynes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2003,4,11]]},"reference":[{"key":"e_1_2_1_2_2","first-page":"291","volume-title":"WHO classification of tumours. 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