Publications by authors named "Willemijn T Quispel"
Article Synopsis
- Langerhans cell histiocytosis (LCH) is a disease that affects the immune system and creates problems with certain blood cells.
- Researchers studied 104 samples of LCH and found special immune structures called tertiary lymphoid structures (TLS) in some of them, which may help the body fight the disease.
- Patients with these TLS had a lower chance of getting more LCH problems, suggesting that the immune system might play an important role in managing LCH.
Langerhans cell histiocytosis (LCH) lesions are characterized by neoplastic CD1a(+)/Langerin(+) histiocytes (LCH-cells) and display many features of chronic inflammation. Cancer cells can escape immune-surveillance through intra-tumoral secretion of immune-suppressive cytokines. We therefore studied by immunohistochemistry the local cytokine milieu and phenotypic characteristics of T-cells and LCH-cells present in LCH lesions collected from 25 therapy naïve patients.
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- Langerhans Cell Histiocytosis (LCH) is a health problem where special cells called CD1a histiocytes gather in body tissues, and some of these cells have mutations that make them act differently.
- Scientists looked closely at parts of a system that helps these cells move and grow to see how they affect LCH in patients.
- The study found that a protein called CXCR4 is important for LCH cells to stay in certain body areas and could help predict if the disease will get worse or not.
Langerhans cell histiocytosis (LCH) is now understood to be a neoplastic disease in which over 50% of cases have somatic activating mutations of BRAF. However, the extracellular signal-related (ERK) pathway is activated in all cases including those with wild type BRAF alleles. Here, we applied a targeted massively parallel sequencing panel to 30 LCH samples to test for the presence of additional genetic alterations that might cause ERK pathway activation.
View Article and Find Full Text PDFPurpose: Poly-ostotic Langerhans Cell Histiocytosis (LCH) can be difficult to distinguish clinically and histologically from disseminated infection in manifesting specific subtypes of Mendelian Susceptibility to Mycobacterial Disease (MSMD). In MSMD-patients, dominant negative germline mutations in the IFN-γR1 gene, in particular in exon 6, lead to autosomal dominant IFN-γ receptor 1 deficiency (ADIFNGR1) and can mimic LCH. We hypothesized that similar defects might underlie the pathogenesis of LCH.
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