杨瑜教授-淋巴瘤诊疗进展.pptVIP

* GCB,ABC和PMBCL源自不同的B细胞分化阶段,不同的发病机制。PMBCL具有PDL2高表达，肿瘤细胞获得性免疫豁免机制，以及JAK-STAT通路和 NF-kB 通路的活化，凋亡受阻。 In the ABC type, the nuclear factor kappa B (NF-κB) pathway is constitutively active, with high expression of NF-κB target genes. Although both GCB and ABC subtypes express B-cell lymphoma 2 (BCL2)—which is induced 30-fold during peripheral B-cell activation—its expression is 4-fold higher in most ABC DLBCLs compared with GCB DLBCLs.[5,6] The distinct genetic characteristics of the GCB and ABC subtypes suggest their derivation from different stages of B-cell differentiation, with the GCB subtype arising from germinal center B cells and the ABC subtype from post–germinal center B cells that are blocked during plasmacytic differentiation * * Shown are the main activation cascades of JAK-STAT and NF-B signaling. Alternative pathway activation exists. Known gene alterations leading to constitutive pathway activity in PMBCL are shown in color. Reprinted from Steidl and Gascoyne9 by permission of the American Society of Hematology. * * * * Representative HE and immunohistochemical stains. Positivity of CD20 and CD30 in PMBCL is characteristic, although not mandatory, for the diagnosis of PMBCL. The HE stain of PMBCL shows typical sheets of medium-sized to large cells containing abundant pale cytoplasm in a background of fine sclerosis. Of note, CD20 staining in nodular sclerosis subtype of cHL is typically heterogeneous, and CD30 is usually absent in DLBCL. (Bottom panel) The typical clinical features of PMBCL are given and contrasted with related entities * MinT试验是针对年龄18-60岁、IPI评分为0/1的低危DLBCL患者的随机前瞻性临床试验，比较RCHOP样方案和CHOP样方案的疗效和安全性。PMBCL患者亚组分析结果显示，在全部入组的824例患者中有87例PMBCL患者，其中43例接受CHOP样治疗、44例接受RCHOP样治疗，大肿块患者化疗后接受放疗。 * * 在87例PMBCL患者中，RCHOP组的CR/CRu率52.3%与32.6%、3年EFS率（78%与52%）优于CHOP组（P=0.012）。两组患者3 年OS率分别为89%和78%（P=0.158）。研究表明，利妥昔单抗显著改善PMBCL患者缓解率和3年EFS率，其生存期接近DLBCL患者。该研究系随机试验中非预先设定的亚组分析结果，存在一定的局限性，可供临床参考 * * R-VACOP-B方案： * * 来自日本的回故性分析 * MACOP-B/VACOP-B方案 vs CHOP方案 一项回顾性分析入组