Glia maturation factor

胶质细胞成熟因子
  • 文章类型: Journal Article
    UNASSIGNED:与经典MF相比,肉芽肿性真菌病(GMF)的预后较差,并且仍然是一个重要的诊断难题。我们分析了临床病理,免疫表型,和GMF的分子特征来开发诊断算法。我们的方法涉及2014年至2020年数据库中GMF患者的回顾性病例系列研究。总共确定了8例患者,其中9例GMF活检。皮肤表现具有可变的临床表型。组织学上,所有病例均表现为非典型CD4+T细胞浸润,50%(n=4),局灶性37.5%(n=3),并且没有25%(n=2)表皮性。在77.8%的活检(n=7)中发现了肉芽肿形成,其中结节样肉芽肿占57.1%(n=4),环状肉芽肿样类型占42.9%(n=3)。在66.7%的活检中(n=6),CD4:CD8比值>4:1,66.6%(n=6)的活检显示CD7表达缺失≥50%.对活检切片进行的T细胞受体基因重排研究在所有活检中均为阳性(n=6),而外周血T细胞受体基因重排研究未发现克隆性.总之,GMF具有微妙或不存在的表皮性和可变的肉芽肿反应;因此,诊断需要多模式方法,我们提出的算法提供了一个框架来解决这个诊断挑战。
    UNASSIGNED: Granulomatous mycosis fungoides (GMF) harbors a worse prognosis compared with classic MF and remains a significant diagnostic dilemma. We analyzed clinicopathologic, immunophenotypic, and molecular characteristics of GMF to develop a diagnostic algorithm. Our methodology involved a retrospective case series study of patients with GMF from our database between 2014 and 2020. A total of 8 patients with 9 biopsies of GMF were identified. Skin manifestations had variable clinical phenotype. Histologically, all cases demonstrated atypical CD4 + T-cell infiltrate with scant in 50% (n = 4), focal 37.5% (n = 3), and absent 25% (n = 2) epidermotropism. Granuloma formation was seen in 77.8% biopsies (n = 7) with sarcoid-type granulomas in 57.1% (n = 4) and granuloma annulare-like type in 42.9% (n = 3). In 66.7% of biopsies (n = 6), the CD4:CD8 ratio was >4:1 and 66.6% (n = 6) of biopsies showed ≥50% loss of CD7 expression. T-cell receptor gene rearrangement studies performed on biopsy sections were positive in all biopsies (n = 6), whereas peripheral blood T-cell receptor gene rearrangement studies did not identify clonality. In conclusion, GMF has subtle or absent epidermotropism and variable granulomatous reaction; thus, the diagnosis requires a multimodal approach, and our proposed algorithm provides a framework to approach this diagnostic challenge.
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