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Neutrophilic Dermatoses Associated with Myeloid Malignancies

机译:与骨髓恶性肿瘤相关的中性皮肤病

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摘要

Neutrophilic dermatoses (ND) are a group of conditions characterized by an aseptic accumulation of polymorphonuclear leukocytes in the skin. Occurrence of ND in association with myeloid malignancies, mainly myelodysplastic syndrome and myelogenous acute leukemia, is not rare and is often associated with a poor prognosis. Recent findings have improved understanding of the pathophysiology of myeloid malignancy-associated ND. We review the clinical spectrum of myeloid malignancy-associated ND with an emphasis on recently identified mechanisms. Myeloid leukemia cells retain the potential for terminal differentiation into polymorphonuclear leukocytes in the skin. Many studies suggest a clonal link between myeloid malignancies and ND. Activation of autoinflammatory pathways (NOD-like receptor family pyrin domain-containing-3, Familial Mediterranean Fever Gene) in the clonal cells of myeloid disorders may also be involved in this setting.
机译:中性皮肤皮(ND)是一组病症,其特征在于皮肤中多核白细胞的无菌积累。 与骨髓恶性肿瘤有关的ND发生,主要是骨髓增强术综合征和髓性急性白血病,并不罕见,通常与预后差有关。 最近的发现改善了对骨髓恶性肿瘤相关ND的病理生理学的理解。 我们审查了骨髓恶性肿瘤相关的ND的临床谱,重点是最近确定的机制。 髓性白血病细胞保留末端分化的可能性,进入皮肤中多核白细胞。 许多研究表明骨髓恶性肿瘤与ND之间的克隆联系。 在该凝固中也可以参与在骨髓紊乱的克隆细胞中的自身炎症途径(Nod样受体家族吡林域吡林域-3,家族性地中海发热基因)。

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