{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T12:49:09Z","timestamp":1785761349311,"version":"3.56.0"},"reference-count":41,"publisher":"BMJ","issue":"8","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,7,22]]},"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:p>\n                    Polycythaemia vera (PV) may rarely evolve into acute erythroid leukaemia (AEL). We herein report a known case of\n                    <jats:italic>JAK2<\/jats:italic>\n                    p. V617F mutated PV presenting with progressive hepatosplenomegaly, abdominothoracic lymphadenopathy, ascites and various haematological derangements. Rapid diagnostic work-up revealed extensive marrow, hepatic and ascitic fluid infiltration by many CD71+, LMO2+ and p53+ proerythroblasts. Next generation sequencing showed\n                    <jats:italic>TP53<\/jats:italic>\n                    ,\n                    <jats:italic>ASXL1<\/jats:italic>\n                    and\n                    <jats:italic>TET2<\/jats:italic>\n                    gene mutations. A chemotherapeutic regimen of ruxolitinib, azacitidine and venetoclax was initiated. A comprehensive review of the English literature emphasises clinical, morphologic, immunophenotypic and genetic characteristics of AEL transformation in PV.\n                  <\/jats:p>","DOI":"10.1136\/bcr-2026-273700","type":"journal-article","created":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T12:05:30Z","timestamp":1785758730000},"page":"e273700","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Acute erythroid leukaemia transformation in polycythaemia vera with leukemic involvement in liver and ascites"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6374-1015","authenticated-orcid":false,"given":"Allison Kaye Lombridas","family":"Pagarigan","sequence":"first","affiliation":[{"name":"Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-0217-7806","authenticated-orcid":false,"given":"Natcha","family":"Sumpansirikul","sequence":"additional","affiliation":[{"name":"Division of Hematology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ekapun","family":"Karoopongse","sequence":"additional","affiliation":[{"name":"Division of Hematology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9724-2692","authenticated-orcid":false,"given":"Sanya","family":"Sukpanichnant","sequence":"additional","affiliation":[{"name":"Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,8,3]]},"reference":[{"key":"2026080305051553000_19.8.e273700.1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-022-01613-1"},{"key":"2026080305051553000_19.8.e273700.2","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1038\/s41408-023-00878-8","article-title":"Blast phase myeloproliferative neoplasm: contemporary review and 2024 treatment algorithm","volume":"13","author":"Tefferi","year":"2023","journal-title":"Blood Cancer J"},{"key":"2026080305051553000_19.8.e273700.3","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2010.194"},{"key":"2026080305051553000_19.8.e273700.4","unstructured":"Jaffe ES , Arber DA . Hematopathology. 3rd edn. Philadelphia: Elsevier Inc, 2025."},{"key":"2026080305051553000_19.8.e273700.5","doi-asserted-by":"publisher","DOI":"10.1182\/BLOOD.2022015850"},{"key":"2026080305051553000_19.8.e273700.6","doi-asserted-by":"crossref","DOI":"10.3390\/ijms25116256","article-title":"Acute Erythroid Leukemia: From Molecular Biology to Clinical Outcomes","volume":"25","author":"Fernandes","year":"2024","journal-title":"Int J Mol Sci"},{"key":"2026080305051553000_19.8.e273700.7","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1056\/NEJM196609012750908","article-title":"Di Guglielmo\u2019s syndrome after polycythemia","volume":"275","author":"Bank","year":"1966","journal-title":"N Engl J Med"},{"key":"2026080305051553000_19.8.e273700.8","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/110.5.590"},{"key":"2026080305051553000_19.8.e273700.9","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.54.8.608"},{"key":"2026080305051553000_19.8.e273700.10","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1007\/BF02982551","article-title":"Myelodysplastic Syndrome Progresses Rapidly Into Erythroleukemia Associated With Synchronous Double Cancers of the Stomach and the Papilla of Vater","volume":"74","author":"Takahashi","year":"2001","journal-title":"Int J Hematol"},{"key":"2026080305051553000_19.8.e273700.11","first-page":"148","article-title":"Acute erythroid neoplastic proliferations. A biological study based on 62 patients","volume":"87","author":"Domingo-Claros","year":"2002","journal-title":"Haematologica"},{"key":"2026080305051553000_19.8.e273700.12","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(77)90123-1"},{"key":"2026080305051553000_19.8.e273700.13","doi-asserted-by":"crossref","first-page":"1636","DOI":"10.1002\/1097-0142(19801001)46:7<1636::AID-CNCR2820460724>3.0.CO;2-O","article-title":"Erythroblastic Transformation in Myeloproliferative Disorders","volume":"46","author":"Neiman","year":"1980","journal-title":"Cancer"},{"key":"2026080305051553000_19.8.e273700.14","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1016\/0165-4608(81)90045-5","article-title":"Erythroblastic transformation of Philadelphia chromosome (Ph1)-positive chronic myelogenous leukemia associated with marked chromosomal rearrangements","volume":"3","author":"Sadamori","year":"1981","journal-title":"Cancer Genet Cytogenet"},{"key":"2026080305051553000_19.8.e273700.15","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1182\/blood.V62.3.591.591","article-title":"Erythroid blast crisis in chronic myelogenous leukemia","volume":"62","author":"Ekblom","year":"1983","journal-title":"Blood"},{"key":"2026080305051553000_19.8.e273700.16","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1034\/j.1600-0609.2000.90253.x","article-title":"Triple Philadelphia chromosomes with major\u2010 bcr rearrangement in hypotriploid erythroleukaemia","volume":"65","author":"Yamamoto","year":"2000","journal-title":"European J Haematol"},{"key":"2026080305051553000_19.8.e273700.17","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/S0165-4608(02)00574-5","article-title":"Identical abnormality of the short arm of chromosome 18 in two Philadelphia-positive chronic myelocytic leukemia patients with erythroblastic transformation, resulting in duplication of BCR-ABL1 fusion","volume":"138","author":"Khac","year":"2002","journal-title":"Cancer Genet Cytogenet"},{"key":"2026080305051553000_19.8.e273700.18","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/j.cancergencyto.2005.06.010","article-title":"Extra BCR\/ABL fusion mosaicism in 18p in a CML Ph-positive patient with erythroblastic transformation","volume":"165","author":"Vargas","year":"2006","journal-title":"Cancer Genet Cytogenet"},{"key":"2026080305051553000_19.8.e273700.19","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.5858\/2008-132-1021-CMAECM","article-title":"Concurrent Megakaryocytic and Erythroid Chronic Myelogenous Leukemia Blast Crisis","volume":"132","author":"Westfall","year":"2008","journal-title":"Arch Pathol Lab Med"},{"key":"2026080305051553000_19.8.e273700.20","doi-asserted-by":"publisher","DOI":"10.1136\/jclinpath-2014-202740"},{"key":"2026080305051553000_19.8.e273700.21","first-page":"18","article-title":"Erythroid blast crisis in chronic myelogenous leukemia: Case report and review of literature","volume":"5","author":"Nagamos","year":"2016","journal-title":"Leuk Res Rep"},{"key":"2026080305051553000_19.8.e273700.22","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1080\/10428194.2021.1975191","article-title":"Chronic myeloid leukemia with pure erythroid leukemia blast crisis","volume":"63","author":"Jacobs","year":"2022","journal-title":"Leuk Lymphoma"},{"key":"2026080305051553000_19.8.e273700.23","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/aqw033"},{"key":"2026080305051553000_19.8.e273700.24","article-title":"Transformation of Polycythemia Vera to Pure Erythroid Leukemia","volume":"13","author":"Liu","year":"2021","journal-title":"Cureus"},{"key":"2026080305051553000_19.8.e273700.25","doi-asserted-by":"crossref","first-page":"200460","DOI":"10.1016\/j.ehpc.2020.200460","article-title":"An unusual presentation of blast phase in JAK 2 mutated polycythemia vera","volume":"23","author":"Abraham","year":"2021","journal-title":"Hum Pathol Case Rep"},{"key":"2026080305051553000_19.8.e273700.26","doi-asserted-by":"crossref","DOI":"10.1182\/blood.2022015438","article-title":"Pure erythroid leukemia evolved from JAK2V617F-positive polycythemia vera: blast phase transformation along erythroid line","volume":"139","author":"Zhao","year":"2022","journal-title":"Blood"},{"key":"2026080305051553000_19.8.e273700.27","doi-asserted-by":"crossref","DOI":"10.1097\/HS9.0000000000000411","article-title":"Atypical Presentation of Erythroid\/Megakaryocytic Leukemic Transformation of a Myeloproliferative Neoplasm Associated with Mutation and Loss of TP53","volume":"4","author":"Chernak","year":"2020","journal-title":"HemaSphere"},{"key":"2026080305051553000_19.8.e273700.28","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1002\/ajh.20164","article-title":"Essential thrombocythemia terminating in pure erythroleukemia","volume":"77","author":"Kreft","year":"2004","journal-title":"American J Hematol"},{"key":"2026080305051553000_19.8.e273700.29","doi-asserted-by":"crossref","first-page":"100716","DOI":"10.1016\/j.modpat.2025.100716","article-title":"Erythroblastic Sarcoma in Adults and Children: Different Pathways to the Same Destination","volume":"38","author":"Fitzpatrick","year":"2025","journal-title":"Mod Pathol"},{"key":"2026080305051553000_19.8.e273700.30","doi-asserted-by":"publisher","DOI":"10.1016\/S0046-8177(84)80254-3"},{"key":"2026080305051553000_19.8.e273700.31","doi-asserted-by":"publisher","DOI":"10.1309\/AJCP1ZA7HYZKAZHS"},{"key":"2026080305051553000_19.8.e273700.32","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.50.6168"},{"key":"2026080305051553000_19.8.e273700.33","doi-asserted-by":"publisher","DOI":"10.1016\/j.leukres.2012.04.009"},{"key":"2026080305051553000_19.8.e273700.34","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2012.03.026"},{"key":"2026080305051553000_19.8.e273700.35","doi-asserted-by":"publisher","DOI":"10.1016\/j.leukres.2011.10.032"},{"key":"2026080305051553000_19.8.e273700.36","doi-asserted-by":"publisher","DOI":"10.1097\/PAT.0000000000000108"},{"key":"2026080305051553000_19.8.e273700.37","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1111\/pin.12835","article-title":"Erythroblastic sarcoma co\u2010existent with myelodysplastic syndrome","volume":"69","author":"Nozuchi","year":"2019","journal-title":"Pathol Int"},{"key":"2026080305051553000_19.8.e273700.38","doi-asserted-by":"crossref","first-page":"e18","DOI":"10.1002\/dc.24540","article-title":"Erythroblastic sarcoma presenting as a unilateral pleural effusion in a patient with myelodysplastic syndrome\/myeloproliferative neoplasm\u2014Case report with cytologic and histologic findings","volume":"48","author":"Kebe Radulovi\u0107","year":"2020","journal-title":"Diagn Cytopathol"},{"key":"2026080305051553000_19.8.e273700.39","doi-asserted-by":"crossref","first-page":"e98","DOI":"10.1111\/bjh.16379","article-title":"Erythroblastic sarcoma with rare PDGFRA rearrangement responding to tyrosine kinase inhibitor therapy","volume":"188","author":"Tomlinson","year":"2020","journal-title":"Br J Haematol"},{"key":"2026080305051553000_19.8.e273700.40","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1007\/s12308-020-00435-3","article-title":"Erythroblastic sarcoma transformation from a chronic myeloid neoplasm with FGFR1 rearrangement presenting as a pleural effusion: a case report","volume":"14","author":"Dalland","year":"2021","journal-title":"J Hematopathol"},{"key":"2026080305051553000_19.8.e273700.41","doi-asserted-by":"crossref","DOI":"10.1182\/blood.2023022625","article-title":"Erythroblastic sarcoma in a liver biopsy","volume":"143","author":"Bakhshi","year":"2024","journal-title":"Blood"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2026-273700","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T12:05:33Z","timestamp":1785758733000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2026-273700"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8]]},"references-count":41,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2026,8,3]]},"published-print":{"date-parts":[[2026,8]]}},"alternative-id":["10.1136\/bcr-2026-273700"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2026-273700","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8]]}}}