School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA.
J Am Acad Dermatol. 2012 Mar;66(3):503-8. doi: 10.1016/j.jaad.2011.06.014. Epub 2011 Oct 17.
Although Sézary syndrome (SS) represents an advanced stage of cutaneous T-cell lymphoma, this diagnosis presents a challenge even for the most experienced dermatologic clinicians. SS is characterized clinically by erythroderma, but can also be identified in the presence of specific histologic and peripheral blood findings. Erythrodermic cutaneous T-cell lymphoma can mimic a number of nonmalignant disorders with erythroderma, including pityriasis rubra pilaris, psoriasis, atopic dermatitis, and graft-versus-host disease. The diagnosis is made even more challenging because the histology of SS is often nonspecific and rarely pathognomonic. As a result, peripheral blood studies in patients with erythroderma are frequently informative in the diagnosis of SS. Peripheral blood abnormalities including elevated CD4/CD8 ratio, aberrant CD26, CD27 and CD7 expression, and T-cell clonality can all be used to help arrive at a diagnosis. This review evaluates current data on the usefulness and limitations of specific peripheral blood markers detected by flow cytometry and T-cell receptor gene rearrangement polymerase chain reaction.
尽管蕈样肉芽肿（SS）代表皮肤 T 细胞淋巴瘤的晚期阶段，但即使是经验最丰富的皮肤科临床医生也很难做出这一诊断。SS 临床上表现为红皮病，但也可以在特定的组织学和外周血发现中识别出来。红皮病性皮肤 T 细胞淋巴瘤可以模仿许多具有红皮病的非恶性疾病，包括棘层松解性皮病、银屑病、特应性皮炎和移植物抗宿主病。由于 SS 的组织学通常是非特异性的，很少具有特征性，因此诊断更加具有挑战性。因此，红皮病患者的外周血研究经常有助于 SS 的诊断。外周血异常包括升高的 CD4/CD8 比值、异常的 CD26、CD27 和 CD7 表达以及 T 细胞克隆性，都可用于帮助做出诊断。本文综述评估了流式细胞术和 T 细胞受体基因重排聚合酶链反应检测的特定外周血标志物的有用性和局限性的现有数据。
