Miao Yuan, Hu Shimin, Lu Xinyan, Li Shaoying, Wang Wei, Medeiros L Jeffrey, Lin Pei
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030; Department of Pathology, The First Affiliated Hospital and the College of Basic Medical Sciences of China Medical University, Shenyang, China 110001.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030.
Hum Pathol. 2016 Dec;58:72-77. doi: 10.1016/j.humpath.2016.07.025. Epub 2016 Aug 18.
Follicular lymphoma with MYC and BCL2 translocations, so-called double-hit follicular lymphoma (DH-FL), is rare. Here, we report the clinicopathological features of 7 cases of DH-FL. All neoplasms had a follicular pattern (1 partially diffuse). Five cases were predominantly low grade, 4 of which had focal (≤20%) grade 3A areas, and 2 cases were of grade 3. All cases were positive for pan-B-cell antigens, CD10, and BCL6; 6 cases were positive for BCL2. Ki-67 was less than or equal to 50% in 6 cases and 90% in 1 grade 3 case. Three patients presented with stage IV disease and 3 had a Follicular Lymphoma International Prognostic Index score of greater than 2. Six patients received immunochemotherapy, and 1 is still under induction therapy with rituximab, ibrutinib, and lenalidomide. Four achieved complete remission and two had a partial response with persistent or refractory disease. The median follow-up time was 25 months (range, 8.5-53.7 months). Two patients treated with standard regimen for follicular lymphoma had relapsed or refractory disease, and 1 died from complications of allogeneic stem cell transplant administered for relapse. In contrast, all 4 patients treated with more intensive regimen for double-hit lymphoma achieved complete remission. In summary, despite predominantly low-grade histology, cases of DH-FL in this study were aggressive and responded better to more intensive than standard treatment regimens, suggesting DH-FL is part of the spectrum of double-hit high-grade lymphoma.
伴有MYC和BCL2易位的滤泡性淋巴瘤，即所谓的双打击滤泡性淋巴瘤（DH-FL），较为罕见。在此，我们报告7例DH-FL的临床病理特征。所有肿瘤均呈滤泡性模式（1例部分为弥漫性）。5例主要为低级别，其中4例有局灶性（≤20%）3A级区域，2例为3级。所有病例的泛B细胞抗原、CD10和BCL6均呈阳性；6例BCL2呈阳性。6例病例的Ki-67小于或等于50%，1例3级病例为90%。3例患者表现为IV期疾病，3例的滤泡性淋巴瘤国际预后指数评分大于2。6例患者接受了免疫化疗，1例仍在接受利妥昔单抗、伊布替尼和来那度胺的诱导治疗。4例实现完全缓解，2例部分缓解但有持续性或难治性疾病。中位随访时间为25个月（范围8.5 - 53.7个月）。2例接受滤泡性淋巴瘤标准方案治疗的患者出现复发或难治性疾病，1例死于为治疗复发而进行的异基因干细胞移植的并发症。相比之下，4例接受更强化方案治疗双打击淋巴瘤的患者均实现完全缓解。总之，尽管本研究中的DH-FL病例主要为低级别组织学，但具有侵袭性，对强化治疗方案的反应优于标准治疗方案，提示DH-FL是双打击高级别淋巴瘤谱系的一部分。
