Open Access Case Report Article ID: AHCRR-7-135

    Combined treatment with vemurafenib and cobimetinib in Langerhans cell histiocytosis and Erdheim-Chester disease overlap syndrome: A case Report

    Hidalgo-Soto Marta*, Poza-Santaella María, Pita-Suárez Daniel, Calbacho-Robles María, Pina-Sánchez José, González-Medina José and Baumann Tycho Stephan

    Histiocytoses are clonal disorders diseases derived from the monocyte-macrophage lineage. The Erdheim–Chester Disease (ECD) and Langerhans Cell Histiocytosis (LCH) may occur in association with overlapping clinical, histopathological and molecular features, harboring somatic MAP2K1 mutations in more than 50% of patients. BRAF and MEK inhibitors have shown to be efficacious in ECD and LCH, including responses in patients with CNS involvement.

    This case report describes a 59-year-old woman who presented with vemurafenib-refractory ECD/LCH overlap syndrome treated with vemurafenib/cobimetinib dual therapy, with rapidly progressing neurological involvement after its initiation.

    Although targeted therapy plays a crucial role in the treatment of histiocytosis, only anecdotal clinical cases treated with dual therapy have been reported in ECD or LCH and collaborative trials are needed to improve outcomes.


    Published on: Jan 20, 2022 Pages: 1-5

    Full Text PDF Full Text HTML DOI: 10.17352/ahcrr.000035
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