{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T08:08:13Z","timestamp":1753862893066,"version":"3.41.2"},"reference-count":6,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2023,8,16]],"date-time":"2023-08-16T00:00:00Z","timestamp":1692144000000},"content-version":"vor","delay-in-days":15,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Case Reports"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:sec><jats:title>Key Clinical Message<\/jats:title><jats:p>This is the first case of a promyelocytic sarcoma diagnosed on pleural effusion and exposed the difficulty of demonstrating a leukemic phase in patients with bone diseases, such as Gorham's disease. It also showed that promyelocytic sarcoma can be treated by ATRA\/ATO\u2010based therapy with an efficient and tolerated response.<\/jats:p><\/jats:sec><jats:sec><jats:title>Abstract<\/jats:title><jats:p>Myeloid sarcoma (MS) is a rare extramedullary tumoral infiltration of immature myeloid cells and can occur in different sites of the body, without leukemic infiltration. A 38\u2010year\u2010old woman patient presented at emergency with a pleural effusion, bicytopenias, and Gorham's disease, a very rare bone disorder. In the following days, she worsened with a chylothorax and pancytopenias. Pleural puncture cytologically revealed promyelocytes with Auer rods. Cytogenetic and molecular analyses subsequently confirmed the presence of the t(15:17) translocation. However, no circulating phase of these atypical promyelocytes was found. Similarly, no other origin was identified. We conclude that the patient had a MS of unknown etiology in the form of a pleural effusion with pathological promyelocytes. The patient was treated with a combination of oral all\u2010trans retinoic acid (ATRA) and arsenic trioxide (ATO) with a cytological and molecular remission persisting 3\u2009months after diagnosis. We report here the first case of a promyelocytic MS of pleural origin without concomitant evidence of acute promyelocytic leukemia. We also show the efficacy of ATRA\/ATO treatment in this etiology.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ccr3.7785","type":"journal-article","created":{"date-parts":[[2023,8,23]],"date-time":"2023-08-23T13:29:57Z","timestamp":1692797397000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["An atypical promyelocytic sarcoma and pleural effusion in a patient with Gorham's disease: Efficiency of <scp>ATRA<\/scp>\/<scp>ATO<\/scp>\u2010based treatment"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5644-926X","authenticated-orcid":false,"given":"Romain","family":"Loyaux","sequence":"first","affiliation":[{"name":"Medical Biology and Patholoy Department Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sol\u00e8ne","family":"Lecolant","sequence":"additional","affiliation":[{"name":"Medical Biology and Patholoy Department Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leila","family":"Cysique\u00a0Foilan","sequence":"additional","affiliation":[{"name":"Hematology Department Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caroline","family":"Pradon","sequence":"additional","affiliation":[{"name":"Medical Biology and Patholoy Department Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sophie","family":"Cotteret","sequence":"additional","affiliation":[{"name":"Medical Biology and Patholoy Department Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean\u2010Baptiste","family":"Micol","sequence":"additional","affiliation":[{"name":"Hematology Department Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annabelle","family":"Stoclin","sequence":"additional","affiliation":[{"name":"Intensive Care Unit Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V\u00e9ronique","family":"Saada","sequence":"additional","affiliation":[{"name":"Medical Biology and Patholoy Department Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christophe","family":"Marzac","sequence":"additional","affiliation":[{"name":"Medical Biology and Patholoy Department Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmadreza","family":"Arbab","sequence":"additional","affiliation":[{"name":"Medical Biology and Patholoy Department Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,8,16]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-022-01613-1"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2404491"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ccr3.1991"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1300874"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.3121\/cmr.3.2.65"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2023.1165091"}],"container-title":["Clinical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ccr3.7785","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,24]],"date-time":"2023-08-24T05:40:05Z","timestamp":1692855605000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ccr3.7785"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,8]]},"references-count":6,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2023,8]]}},"alternative-id":["10.1002\/ccr3.7785"],"URL":"https:\/\/doi.org\/10.1002\/ccr3.7785","archive":["Portico"],"relation":{},"ISSN":["2050-0904","2050-0904"],"issn-type":[{"type":"print","value":"2050-0904"},{"type":"electronic","value":"2050-0904"}],"subject":[],"published":{"date-parts":[[2023,8]]},"assertion":[{"value":"2023-04-21","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-07-03","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-08-16","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e7785"}}