Article
Profound systemic alteration of the immune phenotype and an immunoglobulin switch in Erdheim-Chester disease in 78 patients from a single center.
Haematologica - 1 Jun 2022
Aubart Fleur Cohen, Poupel Lucie, Saint-Charles Flora, Charlotte Frederic, Arsafi Youssef, Frisdal Eric, Roos-Weil Damien, Emile Jean-Francois, Amoura Zahir, Guerin Maryse, Lesnik Philippe, Haroche Julien, Le Goff Wilfried
Abstract excerpt
Erdheim-Chester disease (ECD) is a rare, systemic, non-Langerhans cell histiocytosis neoplasm, which is characterized by the infiltration of CD63+ CD1a- histiocytes in multiple tissues. The BRAFV600E mutation is frequently present in individuals with ECD and has been detected in hematopoietic stem cells and immune cells from the myeloid and systemic compartments. Immune cells and pro-inflammatory cytokines are...
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