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No use, distribution or reproduction is permitted which does not comply with these terms. Most adult histiocytoses are considered clonal diseases because they highlight recurrent somatic mutations in the MAP-kinase pathway gene, primarily BRAF.
Nevertheless, few data are available on adult clonal histiocytosis. This is why we have conducted a retrospective study of all patients with clonal histiocytosis in our institution and present the data according to the presence of BRAF mutation. Those patients had frequent multicentric disease with risk organ involvement, especially the brain and cardiovascular system. They had frequent associated myeloid neoplasms mostly chronic myelomonocytic leukemia and received more frequently targeted therapy as the front-line therapy.
Nevertheless, its presence did not affect the overall survival or relapse-free survival probably due to the emergence of efficient therapies. To conclude, rapid and accurate molecular establishment in adult clonal histiocytoses is crucial because BRAF VE mutation correlates with multicentric disease with organ involvement and incomplete metabolic response. Interestingly the genetic landscape analysis of various cancers, such as melanoma, colon cancer, lung cancer, and more recently histiocytic neoplasms, has highlighted the pivotal role of BRAF VE gene, which is considered an important cornerstone in the development of human cancer.
Its presence is associated with disseminated disease, neurodegeneration, and resistance to front-line therapy in pediatric LCH patients 6 , 7. Its presence is significant because patients with cardiac and neurological locations are frequently BRAF -mutated 8.
