ArticleBlood advances2024
Indeterminate DC histiocytosis is distinct from LCH and often associated with other hematopoietic neoplasms.
Article in Blood advances, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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14 citing papers in PubMed.
- In vitro differentiation and phenotypic profiling of Langerhans cell subsets derived from human peripheral blood monocytes.Immunologic research · 2026Article
- Genomic and clinicopathologic analysis of conventional dendritic cell sarcomas coexisting with myeloid neoplasms.Virchows Archiv : an international journal of pathology · 2026Article
- Histiocytosis development and clinical variation through the lens of genomics.The Journal of pathology · 2026Review
- The histopathological differential diagnosis of BPDCN and other plasmacytoid dendritic cell proliferations.Virchows Archiv : an international journal of pathology · 2026Review
- Common ancestral origin of indeterminate dendritic cell tumor and chronic myelomonocytic leukemia in clonal hematopoiesis.Haematologica · 2026Article
- Frequent NPM1 mutation, monoblastic/monocytic origin and prognostic significance of organ and system involvement in myeloid sarcoma: a multicenter study.The journal of pathology. Clinical research · 2026Article
- Transcription factor Etv3 controls the tolerogenic function of dendritic cells.Science (New York, N.Y.) · 2026Article
- Histiocytoses and reactive proliferations of histiocytes: current state of the art and evolving concepts-a report from the joint CSHP-EA4HP-SH workshop 2024, Hefei, China.Virchows Archiv : an international journal of pathology · 2026Review
- Plasmacytoid dendritic cell proliferations, malignant histiocytic (macrophage/dendritic cell) neoplasms and follicular dendritic cell neoplasms: report from the 2024 Joint CSHP/EA4HP/SH workshop.Virchows Archiv : an international journal of pathology · 2025Article
- Histiocyte Society blueprint for non-Langerhans cell histiocytosis research: unraveling complex diseases through collaboration.Haematologica · 2025Review
- NTRK1-rearranged histiocytosis: clinicopathologic and molecular features.Blood advances · 2025Article
- IDCH: is it now determined?Blood advances · 2025Article
- Updates on Langerhans cell histiocytosis and other histiocytosis in children: invited review-challenges and novelties in paediatric tumours.Virchows Archiv : an international journal of pathology · 2025Review
- Indeterminate cell histiocytosis with ETV3::NCOA2 fusion detected by optical genome mapping.Anais brasileiros de dermatologiaArticle
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19 authors.
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Abstract
abstractIndeterminate 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<20% immunophenotypic profile) examining the clinical, pathologic, and molecular landscape. Median age at presentation was 70 years (interquartile range, 44-80) with cutaneous (31/43; 72%) and nodal (11/43; 26%) involvement predominating. Eighteen (42%) individuals had an associated nonhistiocytic hematopoietic neoplasm ("secondary" IDCH) whereas 7 of 43 (16%) had a concurrent non-IDCH histiocytosis ("mixed" histiocytosis). Most cases exhibited morphology indistinguishable from LCH but with a CD1c+/CSF1R(CD115)- phenotype, mirroring the signature of normal indeterminate cells and conventional DC type 2. Mutational analysis revealed frequent KRAS (13/32; 41%) and BRAF p.V600E (11/36, 31%) mutations that were nearly mutually exclusive. RNA-sequencing analysis uncovered ETV3::NCOA2 fusion in 6 other patients presenting as a sole genetic alteration without any other concurrent histiocytic or hematopoietic neoplasm. BRAF and MAP2K1 alterations were significantly associated with partial/retained (1%-20%) Langerin expression (P = .005) and mixed histiocytosis (P = .002). Remarkably, myeloid alterations (DNMT3A, TET2, and SRSF2) co-occurred in IDCH tissues of several individuals. Paired sequencing of IDCH and concurrent non-IDCH hematopoietic neoplasm in 4 individuals revealed shared mutations. Age at diagnosis and any nodal involvement at diagnosis predicted inferior overall survival, but BRAF/RAS pathway alterations did not affect outcome. These data have implications for the diagnostic evaluation, classification, and therapeutic management of IDCH.
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