Vemurafenib for BRAF V600‐mutant Erdheim–Chester disease presenting with bilateral orbital involvement

ECD is considered to have rapid progression and poor prognosis. Studies have shown that vemurafenib is effective for ECD patients with orbital involvement, but not for ECD with multiple organs. The refinement of treatment approaches and the increased awareness of ECD have led to a dramatic improvement in prognosis.

[1]  S. Fabara,et al.  Vemurafenib in the Treatment of Erdheim Chester Disease: A Systematic Review , 2022, Cureus.

[2]  A. Goodman,et al.  Erdheim-Chester disease: Consensus recommendations for the evaluation, diagnosis, and treatment in the molecular era. , 2020, Blood.

[3]  Z. Amoura,et al.  Erdheim-Chester disease. , 2020, Blood.

[4]  Ryanne A. Brown,et al.  Orbital and chorioretinal manifestations of Erdheim-Chester disease treated with vemurafenib , 2018, American journal of ophthalmology case reports.

[5]  S. Ascani,et al.  Erdheim–Chester disease: description of two illustrative cases involving the lung , 2018, Histopathology.

[6]  E. Jaffe,et al.  The clinical spectrum of Erdheim-Chester disease: an observational cohort study. , 2017, Blood advances.

[7]  K. Richani,et al.  Erdheim-Chester disease with orbital involvement: Case report and ophthalmic literature review , 2016, Orbit.

[8]  A. Fischer,et al.  Revised Classification of Histiocytoses and Neoplasms of the Macrophage-dendritic Cell Lineages Histiocyte and Dendritic Cell Lineages the " L " (langerhans) Group , 2022 .

[9]  V. Meignin,et al.  Association of both Langerhans cell histiocytosis and Erdheim-Chester disease linked to the BRAFV600E mutation. , 2014, Blood.

[10]  O. Abdel-Wahab,et al.  Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. , 2014, Blood.

[11]  D. Dormont,et al.  Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings. , 2010, Radiology.

[12]  J. Piette,et al.  Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature , 2006, Journal of Neurology.

[13]  Dr. William Chester.  Über Lipoidgranulomatose , 2005, Virchows Archiv für pathologische Anatomie und Physiologie und für klinische Medizin.