Combined treatment with vemurafenib and cobimetinib in Langerhans cell histiocytosis and Erdheim-Chester disease overlap syndrome: A case Report
<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 mutation...
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| Main Authors: | , , , , , , |
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| Format: | Book |
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Archives of Hematology Case Reports and Reviews - Peertechz Publications,
2022-01-20.
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| Online Access: | Connect to this object online. |
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| Summary: | <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> |
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| DOI: | 10.17352/ahcrr.000035 |