González-Guerrero Laura, Castellet Helena, Martínez Clara, González Nuria, Guijarro Francesca, Lloveras Natalia, Pratcorona Marta, Gich Ignasi, Berenguer-Molins Pau, Perera-Bel Júlia, Zamora Lurdes, Mascaró Martí, Sampol Antonia, Garcia-Guiñón Antoni, Vives Susana, Tormo Mar, Arnan Montserrat, Villamor Neus, Nomdedéu Josep F
Department of Hematology, Hospital de la Santa Creu i Sant Pau. Universitat Autònoma de Barcelona. IIB Sant Pau, Institut Josep Carreras, Sant Quintí, 89, Barcelona, 08041, Spain.
Unitat d'Hematopatologia, Servei d'Anatomia Patológica, Hospital Clínic. IDIBAPS, Barcelona, Spain.
Diagn Pathol. 2025 Apr 30;20(1):56. doi: 10.1186/s13000-025-01655-w.
CD200 is a glycoprotein that binds with its receptor CD200R, providing immunosuppressive signals to T and NK cells. CD200 is expressed by normal stem cells and progenitors committed to B-lymphopoiesis and myeloid development. CD200 biological relevance in acute leukemias is only partially understood.The study included a consecutive series of four hundred thirty-one patients with acute myeloid leukemia (AML). Immunophenotype was established by multiparametric flow cytometry, and the genetic diagnosis was performed by PCR-based methods and a targeted resequencing method covering 42 genes.66% of AML patients expressed CD200 being significantly associated with CD34 reactivity. The frequency of CD200 positivity was higher in cases with core-binding factor genetic lesions such as RUNX1-RUNX1T1 (81.3%) fusions and CBFB-MHY11 (63.2%) rearrangements and also with biallelic CEBPA mutations (100%). The molecular AML group with the lowest CD200 reactivity (19.1%) corresponded to AML with NPM1 mutations. RNA seq showed no uniform pattern of infiltrating cells in CEBPA mutated AML. Deconvolution analysis may be used to assess the immunoregulatory mechanisms of AML.CD200 expression could help identify the more immature compartment and, combined with other markers, single out CEPA-mutated AML.
CD200是一种糖蛋白,它与其受体CD200R结合,向T细胞和自然杀伤(NK)细胞提供免疫抑制信号。CD200由致力于B淋巴细胞生成和髓系发育的正常干细胞和祖细胞表达。CD200在急性白血病中的生物学相关性仅得到部分了解。该研究纳入了连续的431例急性髓系白血病(AML)患者。通过多参数流式细胞术确定免疫表型,并通过基于聚合酶链反应(PCR)的方法和覆盖42个基因的靶向重测序方法进行基因诊断。66%的AML患者表达CD200,这与CD34反应性显著相关。在伴有核心结合因子基因病变的病例中,如RUNX1-RUNX1T1(81.3%)融合和CBFB-MHY11(63.2%)重排,以及双等位基因CEBPA突变(100%)的病例中,CD200阳性频率更高。CD200反应性最低的分子AML组(19.1%)对应于伴有NPM1突变的AML。RNA测序显示,CEBPA突变的AML中浸润细胞没有统一的模式。去卷积分析可用于评估AML的免疫调节机制。CD200表达有助于识别更不成熟的细胞区室,并与其他标志物结合,筛选出CEPA突变的AML。