Garnache-Ottou Francine, Feuillard Jean, Saas Philippe
EFS BFC, Haematology and Immunology Laboratory, Besançon, France.
Br J Haematol. 2007 Feb;136(4):539-48. doi: 10.1111/j.1365-2141.2006.06458.x.
CD4(+)/CD56(+) haematodermic neoplasm or 'early' plasmacytoid dendritic cell leukaemia/lymphoma (pDCL) was described as a disease entity in the last World Health Organisation/European Organisation for Research and Treatment of Cancer classification for cutaneous lymphomas. These leukaemia/lymphomas co-express CD4 and CD56 without any other lineage-specific markers and have been identified as arising from plasmacytoid dendritic cells. Despite a fairly homogeneous pattern of markers expressed by most pDCL, numerous distinctive features (e.g. cytological aspects and aberrant marker expression) have been reported. This may be related to the 'lineage-independent developmental' programme of dendritic cells, which may be able to develop from either immature or already committed haematopoietic progenitors. This highlights the need for specific validated markers to diagnose such aggressive leukaemia. Here, we propose--among others (e.g. T-cell leukaemia 1)--blood dendritic cell antigen-2 and high levels of CD123 expression as potential markers. In addition, we propose a multidisciplinary approach including several fields of haematology to improve pDCL diagnosis.
CD4(+)/CD56(+)血液肿瘤或“早期”浆细胞样树突状细胞白血病/淋巴瘤（pDCL）在上一版世界卫生组织/欧洲癌症研究与治疗组织皮肤淋巴瘤分类中被描述为一种疾病实体。这些白血病/淋巴瘤共表达CD4和CD56，无任何其他谱系特异性标志物，已被确定起源于浆细胞样树突状细胞。尽管大多数pDCL表达的标志物模式相当一致，但已有许多独特特征（如细胞学特征和异常标志物表达）的报道。这可能与树突状细胞的“不依赖谱系的发育”程序有关，该程序可能能够从未成熟或已定向的造血祖细胞发育而来。这凸显了需要特定的经过验证的标志物来诊断这种侵袭性白血病。在此，我们提出——除其他标志物（如T细胞白血病1）外——血液树突状细胞抗原-2和高水平的CD123表达作为潜在标志物。此外，我们提出一种多学科方法，包括血液学的多个领域，以改善pDCL的诊断。
