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International experts set out how to diagnose Erdheim-Chester disease, which scans and mutation tests to do, and when to use BRAF and MEK inhibitors, interferon or other drugs.
Consensus recommendations from an international group of histiocytosis specialists covering the clinical features, imaging (whole-body PET-CT, cardiac and brain MRI), biopsy and molecular testing (BRAF V600E and other MAPK pathway alterations), the decision to treat, and therapy: BRAF inhibitors for BRAF-mutant disease, MEK inhibitors for BRAF wild-type disease, interferon alfa and other options, with guidance on monitoring and long-term management.
It updates the 2014 recommendations for the targeted therapy era.
The diagnostic and treatment rows on the Erdheim-Chester page follow these recommendations.
Shares Erdheim-Chester disease, Vemurafenib.
Shares Cobimetinib, Vemurafenib.
Shares Erdheim-Chester disease, Blood.
Shares Cobimetinib, Blood.
Shares Cobimetinib, Vemurafenib.
Shares Cobimetinib, Vemurafenib.
Shares Cobimetinib, Vemurafenib.
Shares Interferon alfa-2a/2b, Erdheim-Chester disease, Cobimetinib, Vemurafenib.