{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T01:42:43Z","timestamp":1792028563792,"version":"4.4.0"},"reference-count":58,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2022,4,26]],"date-time":"2022-04-26T00:00:00Z","timestamp":1650931200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Kaplan Cancer Research Fund"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,8,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>This review summarizes the case studies of PCM1-JAK2 fusion tyrosine kinase gene-related neoplasia. Recommended treatment includes JAK2 inhibitors and hematologic stem cell transplantation (HSCT), although the small number of patients has limited study of their efficacy. Herein, we present all available cases in the current searchable literature with their demographics, diagnoses, treatments, and outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>PubMed, ScienceDirect, Publons, the Cochrane Library, and Google were searched with the following terms: PCM1-JAK2, ruxolitinib and myeloid\/lymphoid.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty-six patients (mean age = 50, 77% male) had an initial diagnosis of myeloproliferative neoplasm (MPN) in 40, acute leukemia in 21 and T-cell cutaneous lymphoma in 5. Thirty-five patients (53%) had completed 5-year follow-up. The 5-year survival for the MPN, acute myelogenous leukemia (AML), acute lymphocytic leukemia, and lymphoma groups are 62.7, 14.9%, 40.0%, and 100%, respectively. Too few patients have been treated with ruxolitinib to draw conclusions regarding its effect on survival while the 5-year survival for MPN patients with or without HSCT was 80.2% (40.3%-94.8%) versus 51.5% (22.3%-74.6%), respectively. The T-cell cutaneous lymphoma patients have all survived at least 7 years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This rare condition may be increasingly detected with wider use of genomics. Ruxolitinib can yield hematologic and molecular remissions. However, HSCT is, at this time, the only potentially curative treatment. Useful prognostic markers are needed to determine appropriate timing for HSCT in patients with MPN. Patients presenting with acute leukemia have a poor prognosis.<\/jats:p><\/jats:sec>","DOI":"10.1093\/oncolo\/oyac072","type":"journal-article","created":{"date-parts":[[2022,3,23]],"date-time":"2022-03-23T20:11:34Z","timestamp":1648066294000},"page":"e661-e670","source":"Crossref","is-referenced-by-count":20,"title":["<i>PCM1-JAK2<\/i>Fusion Tyrosine Kinase Gene-Related Neoplasia: A Systematic Review of the Clinical Literature"],"prefix":"10.1093","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9866-5200","authenticated-orcid":false,"given":"Henry G","family":"Kaplan","sequence":"first","affiliation":[{"name":"Swedish Cancer Institute , Seattle, WA , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruyun","family":"Jin","sequence":"additional","affiliation":[{"name":"Center for Cardiovascular Analytics, Research and Data Science (CARDS), Providence Heart Institute, Providence Research Network , Portland, OR , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlo B","family":"Bifulco","sequence":"additional","affiliation":[{"name":"Providence St Joseph Health System , Portland, OR , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James M","family":"Scanlan","sequence":"additional","affiliation":[{"name":"Swedish Center for Research and Innovation , Seattle, WA , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David R","family":"Corwin","sequence":"additional","affiliation":[{"name":"CellNetix , Seattle, WA , USA"},{"name":"Swedish Medical Center , Seattle, WA , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,4,26]]},"reference":[{"key":"2022080910400163100_CIT0001","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1179\/102453309X12473408860226","article-title":"Clinical significance of V617F mutation of the JAK2 gene in patients with chronic 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