{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,3,21]],"date-time":"2023-03-21T05:11:15Z","timestamp":1679375475755},"reference-count":17,"publisher":"Georg Thieme Verlag KG","issue":"07","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dtsch Med Wochenschr"],"published-print":{"date-parts":[[2023,3]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>\n          Anamnese und klinischer Befund Ein 40-j\u00e4hriger, bislang gesunder Patient stellte sich mit neu aufgetretener, h\u00e4morrhagischer Diathese auf unserer Notfallstation vor. Klinisch fanden sich ausgepr\u00e4gte Blutungsstigmata mit gro\u00dffl\u00e4chigen Ekchymosen im Bereich der Oberschenkel sowie enorale Schleimhauteinblutungen, bei sonst allgemeinem Wohlbefinden.<\/jats:p><jats:p>\n          Untersuchungen Die durchgef\u00fchrte Gerinnungsdiagnostik war mit dem Bild einer disseminierten intravasalen Verbrauchskoagulopathie vereinbar. Im mikroskopischen Blutbild fand sich zudem eine leuk\u00e4mische Ausschwemmung von 74% morphologisch atypischen Promyelozyten.<\/jats:p><jats:p>\n          Diagnose, Therapie und Verlauf Die in der Folge durchgef\u00fchrte Knochenmarkdiagnostik stellte die Diagnose einer akuten Promyelozytenleuk\u00e4mie (mikrogranul\u00e4rer Variante). Neben der Gerinnungsoptimierung wurde noch vor Diagnosebest\u00e4tigung auf der Notfallstation eine Therapie mit der Vitamin-A-S\u00e4ure Tretinoin (engl. all-trans retinoic acid, ATRA) eingeleitet, welche im Verlauf um Arsentrioxid (ATO) und das Anthrazyklin Idarubicin erg\u00e4nzt wurde. Erfreulicherweise zeigte sich ein komplikationsarmer weiterer Verlauf. Der Patient ist zudem hinsichtlich der akuten Promyelozytenleuk\u00e4mie aktuell in kompletter Remission.<\/jats:p><jats:p>\n          Folgerungen Die akute Promyelozytenleuk\u00e4mie macht etwa 10\u201315% aller akuten myeloischen Leuk\u00e4mien aus, geht initial h\u00e4ufig mit einer ausgepr\u00e4gten Gerinnungsaktivierung im Sinne einer disseminierten intravasalen Verbrauchskoagulopathie einher und verl\u00e4uft unbehandelt rasch t\u00f6dlich. Eine rasche und bereits bei Diagnoseverdacht eingeleitete Therapie mit ATRA, wie auch eine Gerinnungsoptimierung, sind prognostisch entscheidend.<\/jats:p>","DOI":"10.1055\/a-2038-9745","type":"journal-article","created":{"date-parts":[[2023,3,20]],"date-time":"2023-03-20T23:36:35Z","timestamp":1679355395000},"page":"396-399","source":"Crossref","is-referenced-by-count":0,"title":["Teufel mit Engelsfl\u00fcgeln \u2013 wenn Vitamin A Leben rettet"],"prefix":"10.1055","volume":"148","author":[{"given":"Tobias Matthieu","family":"Benoit","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Medizinische Onkologie und H\u00e4matologie, Universit\u00e4tsspital Z\u00fcrich, Z\u00fcrich, Schweiz"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefan","family":"Gundermann","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Medizinische Onkologie und H\u00e4matologie, Universit\u00e4tsspital Z\u00fcrich, Z\u00fcrich, Schweiz"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2023,3,20]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"e2011067","DOI":"10.4084\/MJHID.2011.067","article-title":"History of acute promyelocytic leukemia: a tale of endless revolution","volume":"3","author":"F Lo-Coco","year":"2011","journal-title":"Mediterr J Hematol Infect Dis"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"1630","DOI":"10.1182\/blood-2019-01-894980","article-title":"Management of acute promyelocytic leukemia: updated recommendations from an expert panel of the European LeukemiaNet","volume":"133","author":"MA Sanz","year":"2019","journal-title":"Blood"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1007\/s40487-018-0091-5","article-title":"Acute Promyelocytic Leukemia: A History over 60 Years-From the Most Malignant to the most Curable Form of Acute Leukemia","volume":"7","author":"X Thomas","year":"2019","journal-title":"Oncol Ther"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"1528","DOI":"10.3324\/haematol.2021.280093","article-title":"A risk score based on real-world data to predict early death in acute promyelocytic leukemia","volume":"107","author":"A Osterroos","year":"2022","journal-title":"Haematologica"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"1570","DOI":"10.1182\/blood-2012-02-410746","article-title":"All-trans-retinoic acid, idarubicin, and IV arsenic trioxide as initial therapy in acute promyelocytic leukemia (APML4)","volume":"120","author":"HJ Iland","year":"2012","journal-title":"Blood"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"3171","DOI":"10.1182\/blood-2010-03-276196","article-title":"Front-line treatment of acute promyelocytic leukemia with AIDA induction followed by risk-adapted consolidation for adults younger than 61 years: results of the AIDA-2000 trial of the GIMEMA Group","volume":"116","author":"F Lo-Coco","year":"2010","journal-title":"Blood"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"391","DOI":"10.1182\/asheducation-2008.1.391","article-title":"Curative strategies in acute promyelocytic leukemia","author":"MS Tallman","year":"2008","journal-title":"Hematology Am Soc Hematol Educ Program"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"5650","DOI":"10.1182\/blood-2010-06-288613","article-title":"Does microgranular variant morphology of acute promyelocytic leukemia independently predict a less favorable outcome compared with classical M3 APL? 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