PDF(Pubmed)
Abstract：
Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin lymphoma. Patients with hemophagocytic lymphohistiocytosis (HLH)-associated IVLBCL variants exhibit significantly poor survival. Cytokines play pivotal roles in malignancy-associated HLH as well as in capillary leak syndrome (CLS). The pathogenesis of CLS involves hyperpermeability and transient endothelial dysfunction. Here, we report the first case of HLH-associated IVLBCL variant complicated with CLS. The patient presented with fever, refractory hypoproteinemia, hypotension and severe edema, followed by telangiectasias. Treatment with etoposide and dexamethasone and hydroxyethyl starch-based artificial colloid led to transient improvement. The diagnosis of IVLBCL was confirmed after the sixth bone marrow biopsy. Subsequently, the R-CHOP (rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisolone) regimen was administered and resulted in prompt alleviation of CLS and HLH symptoms. The patient has survived for more than 6 years after combination of immunochemotherapy and autologous peripheral stem-cell transplantation. This case provides some insights into the mechanism and clinical management of IVLBCL complicated with HLH and CLS. Similar cases concerning lymphoma-associated CLSs were also reviewed.
摘要：
血管内大B细胞淋巴瘤（IVLBCL）是非霍奇金淋巴瘤的一种罕见亚型。噬血细胞性淋巴组织细胞增生症（HLH）相关的IVLBCL变异患者的生存率明显较差。细胞因子在恶性肿瘤相关HLH和毛细血管渗漏综合征（CLS）中起关键作用。CLS的发病机制涉及高通透性和短暂性内皮功能障碍。这里,我们报告了首例HLH相关IVLBCL变异并发CLS的病例。病人出现发烧，难治性低蛋白血症,低血压和严重水肿，其次是毛细血管扩张。用依托泊苷和地塞米松和羟乙基淀粉基人工胶体治疗导致短暂的改善。第6次骨髓活检后确诊为IVLBCL。随后，R-CHOP（利妥昔单抗，环磷酰胺,羟基柔红霉素,长春新碱,和泼尼松龙）方案，并导致CLS和HLH症状的迅速缓解。免疫化疗和自体外周干细胞移植联合治疗后，患者存活了6年以上。该病例为IVLBCL合并HLH和CLS的发病机制和临床治疗提供了一些见解。还回顾了与淋巴瘤相关的CLSs有关的类似病例。
