{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T17:00:05Z","timestamp":1788368405418,"version":"build-2803163510"},"reference-count":22,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,1,12]],"date-time":"2024-01-12T00:00:00Z","timestamp":1705017600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005039","name":"Leids Universitair Medisch Centrum","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005039","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2024,4]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>The discovery of somatic genetic alterations established many histiocytic disorders as haematologic neoplasms. We aimed to investigate the demographic characteristics and additional haematologic cancers of patients diagnosed with histiocytic disorders in The Netherlands.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>We retrieved data on histiocytosis patients from the Dutch Nationwide Pathology Databank (Palga). During 1993 to 2022, more than 4000 patients with a pathologist\u2010assigned diagnosis of a histiocytic disorder were registered in Palga. Xanthogranulomas were the most common subtype, challenging the prevailing assumption that Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. LCH and juvenile xanthogranuloma (JXG) had a peak incidence in the first years of life; males were overrepresented among all histiocytosis subgroups. 118 patients had a histiocytic disorder and an additional haematologic malignancy, including 107 (91%) adults at the time of histiocytosis diagnosis. In 16\/118 patients, both entities had been analysed for the same genetic alteration(s). In 11 of these 16 patients, identical genetic alterations had been detected in both haematologic neoplasms. This included two patients with <jats:italic>PAX5<\/jats:italic> p.P80R mutated B cell acute lymphoblastic leukaemia and secondary histiocytic sarcoma, further supporting that <jats:italic>PAX5<\/jats:italic> alterations may predispose (precursor) B cells to differentiate into the myeloid lineage. All 4\/11 patients with myeloid neoplasms as their additional haematologic malignancy\u00a0had shared <jats:italic>N\/KRAS<\/jats:italic> mutations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This population\u2010based study highlights the frequency of xanthogranulomas. Furthermore, our data add to the growing evidence supporting clonal relationships between histiocytic\/dendritic cell neoplasms and additional myeloid or lymphoid malignancies. Particularly adult histiocytosis patients should be carefully evaluated for the development of these associated haematologic cancers.<\/jats:p><\/jats:sec>","DOI":"10.1111\/his.15127","type":"journal-article","created":{"date-parts":[[2024,1,12]],"date-time":"2024-01-12T08:18:56Z","timestamp":1705047536000},"page":"837-846","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Demographics and additional haematologic cancers of patients with histiocytic\/dendritic cell neoplasms"],"prefix":"10.1111","volume":"84","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9793-7032","authenticated-orcid":false,"given":"Paul G","family":"Kemps","sequence":"first","affiliation":[{"name":"Department of Pathology Leiden University Medical Center  Leiden the Netherlands"},{"name":"Princess M\u00e1xima Center for Pediatric Oncology  Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8259-9971","authenticated-orcid":false,"given":"Lennart","family":"Kester","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology  Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8926-1140","authenticated-orcid":false,"given":"Marijn A","family":"Scheijde\u2010Vermeulen","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology  Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7907-7390","authenticated-orcid":false,"given":"Carel J M","family":"van Noesel","sequence":"additional","affiliation":[{"name":"Department of Pathology Amsterdam University Medical Centers  Amsterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1437-214X","authenticated-orcid":false,"given":"Robert M","family":"Verdijk","sequence":"additional","affiliation":[{"name":"Department of Pathology Leiden University Medical Center  Leiden the Netherlands"},{"name":"Department of Pathology Erasmus MC University Medical Center Rotterdam  Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9239-1050","authenticated-orcid":false,"given":"Arjan","family":"Diepstra","sequence":"additional","affiliation":[{"name":"Department of Pathology University Medical Center Groningen  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ari\u00ebnne M W","family":"van Marion","sequence":"additional","affiliation":[{"name":"Department of Pathology VieCuri Medical Center  Venlo the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natasja","family":"Dors","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology  Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6175-6077","authenticated-orcid":false,"given":"Cor","family":"van den Bos","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology  Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1354-6988","authenticated-orcid":false,"given":"Annette H","family":"Bruggink","sequence":"additional","affiliation":[{"name":"Palga (Dutch Nationwide Pathology Databank)  Houten the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1513-8104","authenticated-orcid":false,"given":"Pancras C 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