血管内大B细胞淋巴瘤(IVLBCL)是一种罕见且高度恶性的非霍奇金B细胞淋巴瘤,临床表现少见,预后差。IVLBCL的诊断缺陷主要是由于微妙的组织学变化很容易被忽视,除了罕见的发生,非特异性和可变的临床表现,和没有明显的肿块病变。本研究的目的是进一步探讨IVLBCL的临床病理和分子特征,以确保对该实体的准确诊断。这里,我们回顾性地介绍了4例新病例和文献病例的资料.年龄在23至92岁之间,年龄为67岁,男女比例为1:1。临床表现变化很大,包括发烧,盗汗,减肥,贫血,血小板减少症,原因不明的低氧血症,意识受损,和皮肤损伤,以及极低的血清白蛋白水平,高水平的血清乳酸脱氢酶(LDH),可溶性白细胞介素2受体(sIL2R),和铁蛋白.形态学上,99.9%的病例呈选择性生长模式,小血管腔内的非典型淋巴细胞。此外,绝大多数病例CD20、CD79a阳性,PAX5,MUM1和BCL6以及一部分病例表达BCL2和CD5,而CD3和CD10通常为阴性。Ki-67增殖指数范围为20%至100%。总而言之,我们进行了全面的病例报告,据我们所知,这是报告的最大的IVLBCL病例队列.早期诊断和及时治疗需要全面评估和更多IVLBCL病例。
Intravascular large B-cell lymphoma (IVLBCL) is a rare and highly malignant non-Hodgkin B-cell lymphoma with uncommon clinical presentation and poor prognosis. The diagnostic pitfall of IVLBCL is mainly due to the fact that subtle histological changes could be easily overlooked, in addition to its rare occurrence, non-specific and variable clinical presentations, and the absence of significant mass lesions. The purpose of this
study is to further explore the clinicopathologic and molecular features of IVLBCL to ensure an accurate diagnosis of this entity. Here, we retrospectively present the data of the four new cases and the literature cases. The age ranged from 23 to 92, with a medium age of 67 and a male-to-female ratio of 1:1. The clinical manifestations are extremely variable, including fever, night sweats, weight loss, anemia, thrombocytopenia, unexplained hypoxemia, impaired consciousness, and skin lesions, as well as the extremely low levels of serum albumin, high levels of serum lactate dehydrogenase (LDH), soluble interleukin-2 receptor (sIL2R), and ferritin. Morphologically, 99.9% of cases showed a selective growth pattern with large, atypical lymphocytes within the lumen of small blood vessels. In addition, vast majority of cases were positive for CD20, CD79a, PAX5, MUM1, and BCL6, and a subset of cases expressed BCL2 and CD5, whereas CD3 and CD10 were typically negative. Ki-67 proliferative index ranged from 20% to 100%. To sum up, we have conducted comprehensive case reports, to the best of our knowledge, this is the largest reported cohort of IVLBCL cases. Comprehensive assessments and more IVLBCL cases are required for early diagnosis and prompt treatment.