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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Archives of Hematology Case Reports and Reviews - Peertechz Publications,
2022-01-20.
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| LEADER | 00000 am a22000003u 4500 | ||
|---|---|---|---|
| 001 | peertech__10_17352_ahcrr_000035 | ||
| 042 | |a dc | ||
| 100 | 1 | 0 | |a Hidalgo-Soto Marta |e author |
| 700 | 1 | 0 | |a Poza-Santaella María |e author |
| 700 | 1 | 0 | |a Pita-Suárez Daniel |e author |
| 700 | 1 | 0 | |a Calbacho-Robles María |e author |
| 700 | 1 | 0 | |a Pina-Sánchez José |e author |
| 700 | 1 | 0 | |a González-Medina José |e author |
| 700 | 1 | 0 | |a Baumann Tycho Stephan |e author |
| 245 | 0 | 0 | |a Combined treatment with vemurafenib and cobimetinib in Langerhans cell histiocytosis and Erdheim-Chester disease overlap syndrome: A case Report |
| 260 | |b Archives of Hematology Case Reports and Reviews - Peertechz Publications, |c 2022-01-20. | ||
| 520 | |a <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> | ||
| 540 | |a Copyright © Hidalgo-Soto Marta et al. | ||
| 546 | |a en | ||
| 655 | 7 | |a Case Report |2 local | |
| 856 | 4 | 1 | |u https://doi.org/10.17352/ahcrr.000035 |z Connect to this object online. |