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Sökning: WFRF:(Fischer Cornelius)

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  • Vallecillo-Garcia, Pedro, et al. (författare)
  • A local subset of mesenchymal cells expressing the transcription factor Osr1 orchestrates lymph node initiation
  • 2023
  • Ingår i: Immunity. - : Cell Press. - 1074-7613 .- 1097-4180. ; 56:6, s. 1204-
  • Tidskriftsartikel (refereegranskat)abstract
    • During development, lymph node (LN) initiation is coordinated by lymphoid tissue organizer (LTo) cells that attract lymphoid tissue inducer (LTi) cells at strategic positions within the embryo. The identity and function of LTo cells during the initial attraction of LTi cells remain poorly understood. Using lineage tracing, we demon-strated that a subset of Osr1-expressing cells was mesenchymal LTo progenitors. By investigating the het-erogeneity of Osr1+ cells, we uncovered distinct mesenchymal LTo signatures at diverse anatomical loca-tions, identifying a common progenitor of mesenchymal LTos and LN-associated adipose tissue. Osr1 was essential for LN initiation, driving the commitment of mesenchymal LTo cells independent of neural ret-inoic acid, and for LN-associated lymphatic vasculature assembly. The combined action of chemokines CXCL13 and CCL21 was required for LN initiation. Our results redefine the role and identity of mesenchymal organizer cells and unify current views by proposing a model of cooperative cell function in LN initiation.
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  • Zanotti, Roberta, et al. (författare)
  • Refined diagnostic criteria for bone marrow mastocytosis : a proposal of the European competence network on mastocytosis
  • 2022
  • Ingår i: Leukemia. - : Springer Nature. - 0887-6924 .- 1476-5551. ; 36:2, s. 516-524
  • Tidskriftsartikel (refereegranskat)abstract
    • In the current classification of the World Health Organization (WHO), bone marrow mastocytosis (BMM) is a provisional variant of indolent systemic mastocytosis (ISM) defined by bone marrow involvement and absence of skin lesions. However, no additional diagnostic criteria for BMM have been proposed. Within the registry dataset of the European Competence Network on Mastocytosis, we compared characteristics and outcomes of 390 patients with BMM and 1175 patients with typical ISM. BMM patients were significantly older, predominantly male, had lower tryptase and lower burden of neoplastic mast cells, and displayed a higher frequency of allergic reactions, mainly triggered by Hymenoptera, than patients with typical ISM. The estimated 10-year progression-free survival of BMM and typical ISM was 95.9% and 92.6%, respectively. In BMM patients defined by WHO-based criteria, the presence of one B-Finding and tryptase level >= 125 ng/mL were identified as risk factors for progression in multivariate analyses. BMM patients without any of these risk factors were found to have better progression-free survival (p < 0.05) and better overall survival (p < 0.05) than other ISM patients. These data support the proposal to define BMM as a separate SM variant characterized by SM criteria, absence of skin lesions, absence of B-Findings, and tryptase levels <125 ng/mL.
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