Indeterminate DC histiocytosis is distinct from LCH and often associated with other hematopoietic neoplasms

•IDCH differs from LCH in its clinical, molecular, and immunohistochemical features.•IDCH is often associated with other hematopoietic neoplasms, and such cases are associated with an inferior prognosis. [Display omitted] Indeterminate dendritic cell histiocytosis (IDCH) is a rare and poorly underst...

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Veröffentlicht in:	Blood advances 2024-11, Vol.8 (22), p.5796-5805
Hauptverfasser:	Ozkaya, Neval, Melloul Benizri, Sarah, Venkataraman, Girish, Karai, Laszlo J., Fraitag, Sylvie, Razanamahery, Jérôme, Pittaluga, Stefania, Battistella, Maxime, Pack, Svetlana, Le Pelletier, François, Xi, Liqiang, Moreau, Anne, Lee, Ina, Hélias-Rodzewicz, Zofia, Donadieu, Jean, Haroche, Julien, Raffeld, Mark, Jaffe, Elaine S., Emile, Jean-François
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Aged Aged, 80 and over Dendritic Cells - metabolism Female Hematologic Neoplasms - diagnosis Hematologic Neoplasms - genetics Histiocytosis, Langerhans-Cell - diagnosis Histiocytosis, Langerhans-Cell - genetics Histiocytosis, Langerhans-Cell - pathology Humans Male Middle Aged Mutation
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Zusammenfassung:	•IDCH differs from LCH in its clinical, molecular, and immunohistochemical features.•IDCH is often associated with other hematopoietic neoplasms, and such cases are associated with an inferior prognosis. [Display omitted] Indeterminate dendritic cell histiocytosis (IDCH) is a rare and poorly understood entity characterized by accumulation of CD1a+/S100+ histiocytes (as Langerhans cell histiocytosis [LCH]) but with reduced-absent expression of Langerin/CD207. We assembled 43 cases of IDCH (defined by CD1a+/CD207
ISSN:	2473-9529 2473-9537 2473-9537
DOI:	10.1182/bloodadvances.2024013545