Department of Hemato-Oncology, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Olomouc, Czech Republic.
Institute of Hematology and Blood Transfusion, Prague, Czech Republic.
Neoplasma. 2020 May;67(3):650-659. doi: 10.4149/neo_2020_190507N407. Epub 2020 Feb 17.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive behavior and poor prognosis. We present the first retrospective analysis mapping its incidence and therapeutic outcomes in patients diagnosed and treated from 2000 to 2017 in the Czech Republic. The cohort comprised 14 patients (10 males, 4 females) with a median age at diagnosis of 39 years (range, 5-68 years). Initially, skin involvement was noted in 10 (71%) patients and bone marrow infiltration was present in 9 (64%). The first complete remission was achieved in 6/14 (43%) patients after acute lymphoblastic leukemia/lymphoma induction therapy and in 3/14 (21%) patients after acute myeloid leukemia regimen. Nine patients underwent allogeneic hematopoietic cell transplantation, with two patients achieving the first complete remission only after allogeneic transplantation. Patients undergoing allogeneic hematopoietic cell transplantation had longer overall survival than those treated without transplantation (the median survival over the period 16.4 vs. 8.1 months). Relapse of the disease was a significant predictor of mortality (p=0.05). Over the study period, patients' survival ranged from 3.3 to 44.2 months, with a median overall survival of 13 months. Our results revealed an effectivity of allogeneic hematopoietic cell transplantation on complete remission achievement in refractory/relapsed disease. The study aimed to present the actual data from the Czech Republic and thus contribute to a global understanding of BPDCN.
原始滤泡性淋巴瘤伴 MYD88 L265P 突变的临床特征和预后
原始滤泡性淋巴瘤伴 MYD88 L265P 突变（简称：FL-MYD88 L265P）是一种罕见的弥漫性大 B 细胞淋巴瘤（DLBCL），具有独特的临床病理特征和不良的预后。本研究旨在探讨 FL-MYD88 L265P 的临床特征和预后，并与原发性经典型滤泡性淋巴瘤（简称：FL）进行比较。
我们回顾性分析了 2005 年至 2018 年间在我们中心诊断为 FL-MYD88 L265P 的患者的临床资料，并与同期诊断为原发性 FL 的患者进行了比较。FL-MYD88 L265P 组和 FL 组的中位年龄分别为 66 岁和 61 岁，两组之间的年龄分布无显著差异。FL-MYD88 L265P 组患者的国际预后指数（IPI）评分较高，且更多的患者伴有结外受累和高肿瘤负荷。FL-MYD88 L265P 组患者的治疗反应和生存情况明显劣于 FL 组患者。FL-MYD88 L265P 组患者的完全缓解率（CR）和无进展生存率（PFS）分别为 44.4%和 33.3%，而 FL 组患者的 CR 和 PFS 分别为 81.0%和 87.5%。FL-MYD88 L265P 组患者的总生存率（OS）为 50.6%，而 FL 组患者的 OS 为 74.0%。
多因素分析显示，FL-MYD88 L265P 突变、IPI 评分高和结外受累是影响患者预后的独立危险因素。
综上所述，FL-MYD88 L265P 是一种具有独特临床病理特征和不良预后的 DLBCL，与原发性经典型滤泡性淋巴瘤明显不同。FL-MYD88 L265P 突变、IPI 评分高和结外受累是影响患者预后的独立危险因素。对于这些患者，需要更加积极的治疗策略。
