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

• Главные авторы: Hidalgo-Soto Marta (Автор), Poza-Santaella María (Автор), Pita-Suárez Daniel (Автор), Calbacho-Robles María (Автор), Pina-Sánchez José (Автор), González-Medina José (Автор), Baumann Tycho Stephan (Автор)
• Формат: ‌
• Опубликовано: Archives of Hematology Case Reports and Reviews - Peertechz Publications, 2022-01-20.
• Предметы: Case Report
• Online-ссылка: Connect to this object online.

Итог

<p>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.</p><p>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.</p><p>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.</p>

DOI

10.17352/ahcrr.000035