Mondello Patrizia, Pitini Vincenzo, Barresi Valeria, Brea Elliott Joseph, Di Mirto Cristian, Arrigo Carmela, Cuzzocrea Salvatore, Mian Michael
Department of Human Pathology, University of Messina, Via Consolare Valeria, 98100 Messina, Italy ; Department of Chemical, Biological, Pharmaceutical and Environmental Sciences, University of Messina, Messina, Italy ; Lymphoma Department, Lymphoma Service, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY USA.
Department of Human Pathology, University of Messina, Via Consolare Valeria, 98100 Messina, Italy.
Exp Hematol Oncol. 2016 Jan 6;5:1. doi: 10.1186/s40164-015-0030-1. eCollection 2015.
Although the clinical outcome of patients with diffuse large B cell lymphoma (DLBCL) has been improved by the addition of rituximab to standard chemotherapy, almost one-third fails or relapses after first line treatment. The presence of monoclonal gammopathy (MG) is a known adverse prognostic factor for DLBCL. Because this subset of patients does not benefit from R-CHOP, new therapeutic options are required. Herein, we report the first case of extranodal DBCL of the lung with a concomitant MG who achieved a long lasting complete remission with lenalidomide.
The 73-year-old male patient presented with lateral cervical lymphadenopathy, B symptoms, lactate dehydrogenase and beta2-microglobulin elevation. Computed tomography (CT) showed mediastinal lymphadenopathy and bilateral lung involvement. Biopsy of both disease locations revealed the presence of DLBCL. Successive bone marrow trephine biopsy proved the presence of concordant DLBCL involvement. At the time of diagnosis, a MG was present as well. The patient did not respond to the standard treatments, and subsequently underwent lenalidomide 25 mg/m(2) days 1-21 q28 plus dexamethasone 40 mg days 1-4, 9-12 e 17-20. This therapeutic regimen was efficacious and safe as salvage therapy in extranodal DBCL with a MG. Furthermore, we observed a close association between DLBCL response to therapy and MG levels, suggesting that the amount of M-protein might be a surrogate marker of disease response.
Although DLBCL associated with MG does not respond properly to the standard treatments, it is highly sensitive to lenalidomide, which is why we endorse its role as treatment of choice in this subset of patients. In addition, MG levels appear to correlate with tumor burden, suggesting that it might be a useful marker of disease response. Prospective trials to validate these observations are warranted.
尽管利妥昔单抗联合标准化疗改善了弥漫性大B细胞淋巴瘤（DLBCL）患者的临床结局，但几乎三分之一的患者在一线治疗后仍失败或复发。单克隆丙种球蛋白病（MG）的存在是DLBCL已知的不良预后因素。由于这部分患者无法从R-CHOP方案中获益，因此需要新的治疗选择。在此，我们报告首例伴有MG的肺结外DBCL患者，使用来那度胺后实现了长期完全缓解。
一名73岁男性患者出现颈部外侧淋巴结肿大、B症状、乳酸脱氢酶和β2微球蛋白升高。计算机断层扫描（CT）显示纵隔淋巴结肿大和双侧肺部受累。对两个病变部位进行活检均发现存在DLBCL。连续的骨髓穿刺活检证实存在一致的DLBCL累及。诊断时也存在MG。该患者对标准治疗无反应，随后接受来那度胺25mg/m²，第1 - 21天，每28天一次，联合地塞米松40mg，第1 - 4天、9 - 12天和17 - 20天的治疗。该治疗方案作为伴有MG的结外DBCL挽救治疗有效且安全。此外，我们观察到DLBCL对治疗的反应与MG水平密切相关，提示M蛋白量可能是疾病反应的替代标志物。
尽管与MG相关的DLBCL对标准治疗反应不佳，但对来那度胺高度敏感，这就是为什么我们认可其在这部分患者中作为首选治疗的作用。此外，MG水平似乎与肿瘤负荷相关，提示它可能是疾病反应的有用标志物。有必要进行前瞻性试验以验证这些观察结果。
Zotero 插件
