{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T19:42:03Z","timestamp":1791229323317,"version":"4.1.0"},"reference-count":11,"publisher":"Wiley","license":[{"start":{"date-parts":[[2016,1,1]],"date-time":"2016-01-01T00:00:00Z","timestamp":1451606400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Case Reports in Hematology"],"published-print":{"date-parts":[[2016]]},"abstract":"<jats:p>Various translocations involving the<jats:italic>PDGFRB<\/jats:italic>gene are identified in myeloid neoplasms. However, the<jats:italic>PRKG2\/PDGFRB<\/jats:italic>fusion gene associated with t(4;5)(q21;q33) has previously been reported in only 3 patients. We present the case of a 26-year-old woman with microcytic anemia, basophilia, thrombocytosis, and massive splenomegaly, who was found to have systemic mastocytosis and associated clonal hematological non-mast cell lineage disease (SM-AHNMD), with myeloid neoplasm with<jats:italic>PRKG2\/PDGFRB<\/jats:italic>rearrangement. Initial findings included basophilia (37%, 4.1\u2009k\/<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M1\"><mml:mrow><mml:mi>\u03bc<\/mml:mi><\/mml:mrow><\/mml:math>L), hypercellular marrow with eosinophilia, and increased and atypical megakaryocytes, suggestive of myeloproliferative neoplasm. Additional studies revealed large clusters of CD25 positive mast cells, fulfilling the criteria for the diagnosis of systemic mastocytosis. Consistent with prior reports of this translocation, our patient has responded well to imatinib. This case, in conjunction with others in the literature, suggests a possible connection between t(4;5)(q21;q33)<jats:italic>PRKG2\/PDGFRB<\/jats:italic>and systemic mastocytosis and highlights their favorable response to imatinib.<\/jats:p>","DOI":"10.1155\/2016\/4158567","type":"journal-article","created":{"date-parts":[[2016,8,25]],"date-time":"2016-08-25T17:04:23Z","timestamp":1472144663000},"page":"1-4","source":"Crossref","is-referenced-by-count":4,"title":["A 26-Year-Old Female with Systemic Mastocytosis with Associated Myeloid Neoplasm with Eosinophilia and Abnormalities of<i>PDGFRB<\/i>, t(4;5)(q21;q33)"],"prefix":"10.1155","volume":"2016","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9012-6680","authenticated-orcid":true,"given":"Laura E.","family":"Brown","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KS, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5454-7763","authenticated-orcid":true,"given":"Da","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KS, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diane L.","family":"Persons","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KS, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5293-4620","authenticated-orcid":true,"given":"Abdulraheem","family":"Yacoub","sequence":"additional","affiliation":[{"name":"The Division of Hematologic Malignancies and Cellular Therapeutics, University of Kansas Medical Center, Kansas City, KS, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shivani","family":"Ponnala","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KS, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8177-0538","authenticated-orcid":true,"given":"Wei","family":"Cui","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KS, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","reference":[{"key":"1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cancergencyto.2007.10.021"},{"key":"2","doi-asserted-by":"publisher","DOI":"10.1111\/ijlh.12057"},{"key":"3","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2003.04051.x"},{"key":"4","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23459"},{"key":"5","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2011-0325-rs"},{"key":"6","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.10980"},{"key":"7","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.11836"},{"key":"8","doi-asserted-by":"publisher","DOI":"10.1016\/j.leukres.2008.12.020"},{"key":"9","doi-asserted-by":"publisher","DOI":"10.1006\/bbrc.1996.0477"},{"key":"10","doi-asserted-by":"publisher","DOI":"10.1126\/science.274.5295.2082"},{"key":"11","doi-asserted-by":"publisher","DOI":"10.1159\/000312134"}],"container-title":["Case Reports in Hematology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2016\/4158567.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2016\/4158567.xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2016\/4158567.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2016,8,25]],"date-time":"2016-08-25T17:04:24Z","timestamp":1472144664000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.hindawi.com\/journals\/crihem\/2016\/4158567\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016]]},"references-count":11,"alternative-id":["4158567","4158567"],"URL":"https:\/\/doi.org\/10.1155\/2016\/4158567","relation":{},"ISSN":["2090-6560","2090-6579"],"issn-type":[{"value":"2090-6560","type":"print"},{"value":"2090-6579","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016]]}}}