Demographics and additional haematologic cancers of patients with histiocytic/dendritic cell neoplasms

Paul G. Kemps*, Lennart Kester, Marijn A. Scheijde-Vermeulen, Carel J.M. van Noesel, Robert M. Verdijk, Arjan Diepstra, Ariënne M.W. van Marion, Natasja Dors, Cor van den Bos, Annette H. Bruggink, Pancras C.W. Hogendoorn, Astrid G.S. van Halteren

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Aims: 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. Methods and results: We retrieved data on histiocytosis patients from the Dutch Nationwide Pathology Databank (Palga). During 1993 to 2022, more than 4000 patients with a pathologist-assigned 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 PAX5 p.P80R mutated B cell acute lymphoblastic leukaemia and secondary histiocytic sarcoma, further supporting that PAX5 alterations may predispose (precursor) B cells to differentiate into the myeloid lineage. All 4/11 patients with myeloid neoplasms as their additional haematologic malignancy had shared N/KRAS mutations. Conclusions: This population-based 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.

Original languageEnglish
Pages (from-to)837-846
Number of pages10
Issue number5
Early online date12 Jan 2024
Publication statusPublished - Apr 2024

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© 2024 The Authors. Histopathology published by John Wiley & Sons Ltd.


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