Department of Dermatology, Venereology and Allergy, University Medical Centre Mannheim, Medical Faculty Mannheim, Heidelberg University and Centre of Excellence of Dermatology of Baden-Württemberg, Mannheim, Germany.
European Center of Angioscience, Medical Faculty Mannheim, Heidelberg University, Heidelberg, Germany.
J Dtsch Dermatol Ges. 2019 Mar;17(3):275-285. doi: 10.1111/ddg.13773. Epub 2019 Mar 9.
Primary cutaneous diffuse large B-cell lymphoma, NOS (PCLBCL/NOS) is a rare PCLBCL. Only few data are available for this tumor. The aim of this study was to identify clinical and/or immunohistochemical markers (in addition to Bcl-2) that characterize PCLBCL/NOS, assist in differentiating it from PCLBCL, leg type (PCLBCL/LT) and help to assess the clinical course/prognosis.
Bcl-2 PCLBCL/NOS) cases (n = 14 were compared with Bcl-2 PCLBCL/LT cases (n = 29).
PCLBCL/NOS patients were younger, predominantly male and had better survival rates than patients with PCLBCL/LT. Patients with PCLBCL/NOS presented more often with larger plaques limited to one or two contiguous body regions, whereas PCLBCL/LT cases often presented with disseminated lesions. Neoplastic cells had a higher proliferation rate (Ki67) in PCLBCL/LT patients. The tumor microenvironment of PCLBCL/NOS had a more prominent CD3 infiltrate. Overall survival data for the whole cohort (n = 37) revealed that female gender and Bcl-2 expression correlated with a worse survival rate. Bcl-6 expression and centroblastic subtype correlated with better outcomes. None of the other markers studied (e.g. GCB/non-GCB subtype) correlated with survival rate.
PCLBCL/NOS and PCLBCL/LT differ in their clinical behavior and outcomes. Bcl-2 still seems to be the best marker for discriminating between these two subgroups. Bcl-2, female gender and Bcl-6 represent prognostic markers for PCLBCL.
原发性皮肤弥漫性大 B 细胞淋巴瘤，NOS（PCLBCL/NOS）是一种罕见的 PCLBCL。目前仅有少量数据可用于此类肿瘤。本研究旨在确定可用于辅助区分 PCLBCL/NOS 与 PCLBCL、小腿型（PCLBCL/LT）的临床和/或免疫组化标志物（除 Bcl-2 外），并帮助评估其临床病程/预后。
对 14 例 Bcl-2 阳性 PCLBCL/NOS 病例（PCLBCL/NOS）与 29 例 Bcl-2 阳性 PCLBCL/LT 病例（PCLBCL/LT）进行比较。
PCLBCL/NOS 患者发病年龄更小、以男性为主，且生存率高于 PCLBCL/LT 患者。PCLBCL/NOS 患者常表现为局限于一个或两个相邻体区的较大斑块，而 PCLBCL/LT 患者则常表现为播散性病变。PCLBCL/LT 患者的肿瘤细胞增殖率（Ki67）更高。PCLBCL/NOS 的肿瘤微环境中 CD3 浸润更为明显。对包括 37 例患者的全队列进行总生存数据分析显示，女性性别和 Bcl-2 表达与生存率降低相关。Bcl-6 表达和中心母细胞型与更好的结局相关。研究中未发现其他标志物（如 GCB/非 GCB 亚型）与生存率相关。
PCLBCL/NOS 和 PCLBCL/LT 在临床行为和结局方面存在差异。Bcl-2 似乎仍然是区分这两个亚组的最佳标志物。Bcl-2、女性性别和 Bcl-6 是 PCLBCL 的预后标志物。
