{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T10:04:47Z","timestamp":1753869887414,"version":"3.41.2"},"reference-count":27,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2020,3,2]],"date-time":"2020-03-02T00:00:00Z","timestamp":1583107200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2020,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Essential thrombocythemia (ET) is rare in children, and pediatric guidelines are lacking. Therefore, we aimed to evaluate ET diagnosis and treatment in a pediatric cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>Data of patients with ET from three hospitals were reviewed. Molecular diagnosis included <jats:italic>JAK2<\/jats:italic>V617F, <jats:italic>CALR<\/jats:italic>, and <jats:italic>MPL<\/jats:italic> mutations. Patients were evaluated for acquired von Willebrand syndrome (AVWS). Follow\u2010up included clinical symptoms, adverse events, and treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twelve children (median age: 8 years, range 1\u201014.5) were included. Mean lag period between the first documentation of thrombocytosis until ET diagnosis was 36 months. Six patients were positive for <jats:italic>JAK2<\/jats:italic>V617F and two for <jats:italic>CALR<\/jats:italic> mutations. In six of nine patients, AVWS was diagnosed. At diagnosis, only 33% of patients started therapy with aspirin (<jats:italic>n<\/jats:italic> = 4) and hydroxyurea (<jats:italic>n<\/jats:italic> = 2). In three of eight untreated patients, therapy was added during follow\u2010up. The cohort was followed for a median of 32.5 months (range: 4\u2010108 months). Clinical follow\u2010up disclosed vascular complications in 4 of 12 patients (deep vein thrombosis, <jats:italic>n<\/jats:italic> = 1; transient ischemic attack, <jats:italic>n<\/jats:italic> = 3). Two females experienced excessive bleeding; both were diagnosed with AVWS. Neither leukemia nor myelofibrosis evolved in our cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Increased awareness to pediatric ET is warranted, as delayed diagnosis is common. Compared to adults, AVWS may be more prevalent among children with ET.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.28183","type":"journal-article","created":{"date-parts":[[2020,3,3]],"date-time":"2020-03-03T05:35:58Z","timestamp":1583213758000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Essential thrombocythemia A retrospective case series"],"prefix":"10.1002","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8329-8409","authenticated-orcid":false,"given":"Assaf Arie","family":"Barg","sequence":"first","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"The Israeli National Hemophilia Center Thrombosis Unit and Amalia Biron Research Institute of Thrombosis and Hemostasis Sheba Medical Center  Tel Hashomer Israel"},{"name":"Department of Pediatric Hematology Oncology &amp; BMT The Edmond and Lily Safra Children's Hospital Sheba Medical Center  Ramat\u2010Gan Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amos","family":"Toren","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"Department of Pediatric Hematology Oncology &amp; BMT The Edmond and Lily Safra Children's Hospital Sheba Medical Center  Ramat\u2010Gan Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hannah","family":"Tamary","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"Department of Pediatric Hematology\u2010Oncology Schneider Children's Medical Center of Israel  Petach Tikva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joanne","family":"Yacobovich","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"Department of Pediatric Hematology\u2010Oncology Schneider Children's Medical Center of Israel  Petach Tikva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1680-2388","authenticated-orcid":false,"given":"Orna","family":"Steinberg\u2010Shemer","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"Department of Pediatric Hematology\u2010Oncology Schneider Children's Medical Center of Israel  Petach Tikva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0033-3425","authenticated-orcid":false,"given":"Oded","family":"Gilad","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"Department of Pediatric Hematology\u2010Oncology Schneider Children's Medical Center of Israel  Petach Tikva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gal","family":"Goldstein","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"Department of Pediatric Hematology Oncology &amp; BMT The Edmond and Lily Safra Children's Hospital Sheba Medical Center  Ramat\u2010Gan Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hagit","family":"Miskin","sequence":"additional","affiliation":[{"name":"Pediatric Hematology Oncology Unit Shaare Zedek Medical Center  Jerusalem Israel"},{"name":"Faculty of Medicine Hebrew University  Jerusalem Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shoshana","family":"Revel\u2010Vilk","sequence":"additional","affiliation":[{"name":"Pediatric Hematology Oncology Unit Shaare Zedek Medical Center  Jerusalem Israel"},{"name":"Faculty of Medicine Hebrew University  Jerusalem Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nurit","family":"Rosenbeg","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"The Israeli National Hemophilia Center Thrombosis Unit and Amalia Biron Research Institute of Thrombosis and Hemostasis Sheba Medical Center  Tel Hashomer Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1279-2557","authenticated-orcid":false,"given":"Gili","family":"Kenet","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"The Israeli National Hemophilia Center Thrombosis Unit and Amalia Biron Research Institute of Thrombosis and Hemostasis Sheba Medical Center  Tel Hashomer Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1733-5395","authenticated-orcid":false,"given":"Vered Shkalim","family":"Zemer","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"},{"name":"Clalit Health Services  Petach Tikva Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,3,2]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2404955"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2004.05329.x"},{"key":"e_1_2_10_4_1","first-page":"507","article-title":"Thrombocytosis in children","volume":"63","author":"Chiarello P","year":"2011","journal-title":"Minerva Pediatr"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2000.02239-7.x"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.12002"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-07-101576"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1311347"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-04-018879"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-11-297580"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.15386"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-04-014746"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12185-016-2022-2"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21730"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.3109\/08880018.2012.662712"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-01-761767"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejim.2015.03.009"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.3109\/09537104.2015.1041900"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0b013e3181b1ec9e"},{"key":"e_1_2_10_20_1","doi-asserted-by":"crossref","first-page":"914","DOI":"10.20452\/pamw.3211","article-title":"Prevalence of acquired von Willebrand syndrome during essential thrombocythemia: a retrospective analysis of 170 consecutive patients","volume":"125","author":"Mital A","year":"2015","journal-title":"Pol Arch Med Wewn"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12967"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2013.092684"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-05-643346"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.14443"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2016.146654"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199905063401801"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.2165\/00148581-200709030-00008"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-08-371328"}],"container-title":["Pediatric Blood &amp; 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