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- W2062373783 abstract "Aims Myelofibrosis is the result of aberrant stromal activity which is determined routinely by reticulin staining in bone marrow biopsies. As matrix fibres are the product of activated fibroblasts, we analysed fibre accumulation compared to stromal cell activity during myelofibrosis progression using the fibroblast activation marker platelet‐derived growth factor receptor β ( PDGFR β) by immunohistochemistry. Methods and results Initial and follow‐up bone marrow biopsies from 84 patients with myeloproliferative neoplasia, including 55 cases with primary myelofibrosis, were evaluated from five haematopathology centres. The stromal mass was measured by conventional reticulin staining [myelofibrosis ( MF ) grade, 0–3] and PDGFR β‐positive cells using a novel PDGFR β scoring system (0–3). Results were correlated for prediction of progression. The MF grade and the PDGFR β score showed excellent correlation (Spearman's r = 0.83, P < 0.0001). Elevated PDGFR β scores (higher than MF ‐grade) predicted myelofibrosis progression in total with 43% sensitivity and 57% specificity, and short‐term (within 1 year) progression with 82% sensitivity and 53% specificity. Progression of prefibrotic disease to manifest myelofibrosis could be forecast with 90% sensitivity and 75% specificity. Conclusion PDGFR β highlights stromal cell activation in marrow fibrosis, which is closely related to matrix accumulation, indicating a direct clinical impact especially in prefibrotic myeloproliferative disorders." @default.
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- W2062373783 date "2015-05-20" @default.
- W2062373783 modified "2023-10-18" @default.
- W2062373783 title "Platelet-derived growth factor receptor β (PDGFRβ) immunohistochemistry highlights activated bone marrow stroma and is potentially predictive for fibrosis progression in prefibrotic myeloproliferative neoplasia" @default.
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- W2062373783 doi "https://doi.org/10.1111/his.12704" @default.
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