{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T01:51:27Z","timestamp":1780624287342,"version":"3.54.1"},"reference-count":47,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2022,3,3]],"date-time":"2022-03-03T00:00:00Z","timestamp":1646265600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>Primary myelofibrosis (PMF) is a BCR\/ABL1-negative myeloproliferative neoplasm (MPN) with a shorter overall survival and a higher leukemic transformation than other BCR\/ABL1-negative MPNs. Diagnosis of PMF can be challenging given its clinical, morphologic, molecular overlap with other myeloid neoplasms also associated with myelofibrosis, and reactive conditions.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We summarize and discuss the clinical, morphologic, and molecular features useful for diagnosing PMF as well as salient features helpful in distinguishing PMF from myelodysplastic syndrome with associated fibrosis and autoimmune myelofibrosis using a case-based approach.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>PMF in both its prefibrotic and fibrotic stages, the latter characterized by reticulin\/collagen marrow fibrosis, is characterized by a proliferation of predominantly abnormal megakaryocytes and granulocytes in the bone marrow. Driver mutations in \u00a0 JAK2, CALR, or MPLare seen in approximately 90% of PMF cases. In triple-negative cases, the presence of cytogenetic abnormalities and other somatic mutations identified by next-generation sequencing can help establish a diagnosis of PMF in the appropriate clinical and morphologic context.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Given the significant difference in prognosis and treatment, integration of clinical, morphological, and molecular\/genetic findings is essential in distinguishing PMF from other etiologies that can demonstrate myelofibrosis.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ajcp\/aqac016","type":"journal-article","created":{"date-parts":[[2022,1,14]],"date-time":"2022-01-14T12:07:33Z","timestamp":1642162053000},"page":"518-530","source":"Crossref","is-referenced-by-count":7,"title":["How I Diagnose Primary Myelofibrosis"],"prefix":"10.1093","volume":"157","author":[{"given":"Sonam","family":"Prakash","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine, University of California, San Francisco, San Francisco, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Attilio","family":"Orazi","sequence":"additional","affiliation":[{"name":"Department of Pathology, Texas Tech University Health Sciences Center, El Paso, TX, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,3,3]]},"reference":[{"key":"2022040112230865300_CIT0001","volume-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 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and overt primary myelofibrosis","volume":"129","author":"Guglielmelli","year":"2017","journal-title":"Blood."},{"key":"2022040112230865300_CIT0010","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1002\/ajh.24788","article-title":"Pre-fibrotic\/early primary myelofibrosis vs. WHO-defined essential thrombocythemia: the impact of minor clinical diagnostic criteria on the outcome of the disease","volume":"92","author":"Jeryczynski","year":"2017","journal-title":"Am J Hematol."},{"key":"2022040112230865300_CIT0011","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1182\/bloodadvances.2016000208","article-title":"Targeted deep sequencing in primary myelofibrosis","volume":"1","author":"Tefferi","year":"2016","journal-title":"Blood Adv."},{"key":"2022040112230865300_CIT0012","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1182\/blood-2015-07-661835","article-title":"Whole-exome sequencing identifies novel MPL and JAK2 mutations in triple-negative 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