The DOI matches its Crossref record; 64 of the 64 references it cites were found in a registry or an index.
Langerhans cell histiocytosis
Abstract
Langerhans cell histiocytosis (LCH) is caused by clonal expansion of myeloid precursors that differentiate into CD1a+/CD207+ cells in lesions that leads to a spectrum of organ involvement and dysfunction. The pathogenic cells are defined by constitutive activation of the MAPK signaling pathway. Treatment of LCH is risk-adapted: patients with single lesions may respond well to local treatment, whereas patients with multisystem disease require systemic therapy. Although survival rates for patients without organ dysfunction is excellent, mortality rates for patients with organ dysfunction may reach 20%. Despite progress made in the treatment of LCH, disease reactivation rates remain above 30%, and standard second-line treatment is yet to be established. Treatment failure is associated with increased risks for death and long-term morbidity, including LCH-associated neurodegeneration. Early case series report promising clinical responses in patients with relapsed and refractory LCH treated with BRAF or MEK inhibitors, although potential for this strategy to achieve cure remains uncertain.
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Citations
Cites
Vemurafenib in Multiple Nonmelanoma Cancers with BRAF V600 Mutations
Hyman, D. M., Puzanov, I., Subbiah, V., Faris, J. E., Chau, I., Blay, J.-Y., Wolf, J., Raje, N. S., Diamond, E. L., Hollebecque, A., et al. (2015) · New England Journal of Medicine · doi:10.1056/nejmoa1502309 · Cited by 1,764
Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages
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Self-citationRecurrent BRAF mutations in Langerhans cell histiocytosis
Badalian-Very, G., Vergilio, J.-A., Degar, B. A., MacConaill, L. E., Brandner, B., Calicchio, M. L., Kuo, F. C., Ligon, A. H., Stevenson, K. E., Kehoe, S. M., et al. (2010) · Blood · doi:10.1182/blood-2010-04-279083 · Cited by 1,243
CCR7 governs skin dendritic cell migration under inflammatory and steady-state conditions
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Langerhans cells renew in the skin throughout life under steady-state conditions
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Origin, homeostasis and function of Langerhans cells and other langerin-expressing dendritic cells
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Haupt, R., Minkov, M., Astigarraga, I., Schäfer, E., Nanduri, V., Jubran, R., Egeler, R. M., Janka, G., Micic, D., Rodriguez‐Galindo, C., et al. (2013) · Pediatric Blood & Cancer · doi:10.1002/pbc.24367 · Cited by 692
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Self-citationHaroche, J., Cohen-Aubart, F., Emile, J.-F., Arnaud, L., Maksud, P., Charlotte, F., Cluzel, P., Drier, A., Hervier, B., Benameur, N., et al. (2013) · Blood · doi:10.1182/blood-2012-07-446286 · Cited by 537
Cited by
Goyal, G., Tazi, A., Go, R. S., Rech, K. L., Picarsic, J. L., Vassallo, R., Young, J. R., Cox, C. W., Van Laar, J., Hermiston, M. L., et al. (2022) · Blood · doi:10.1182/blood.2021014343 · Cited by 283
Metadata
- Authors
- Rodriguez-Galindo, C., & Allen, C. E.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2020-04-16
- Volume
- 135
- Issue
- 16
- Pages
- 1319-1331
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W3007428404
- Cited by
- 361
- References
- 64
- FWCI
- 21.75
- Type
- journal article
- Language
- en
- Open access
- closed
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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