Department of Hematology, University Hospital of Montpellier, Montpellier, France.
Department of Hematology, Léon Bérard Center, Lyon, France.
Ann Oncol. 2017 Apr 1;28(4):843-848. doi: 10.1093/annonc/mdw684.
Plasmablastic lymphoma (PBL), initially described in 1997 in the oral cavity of HIV positive patients, is now recognized as a distinct aggressive and rare entity of diffuse large B-cells lymphoma by the World Health Organization (WHO) classification. Since the original description, others cases have been reported. However, these are largely derived from case reports or small series limiting any definitive conclusions on clinical characteristics and outcome.
The clinical, biological, pathological features and outcome of a cohort including 135 patients with PBL, from LYSA centers in France and Belgium, were reported and analyzed.
The median age was 58 years, with a male predominance. The cohort was divided into 56 HIV-positive patients, 17 post-transplant patients and 62 HIV-negative/non-transplanted patients. Within HIV-negative/non-transplanted, a relative immunosuppression was found in most cases (systemic inflammatory disease, history of cancer, increased age associated with weakened immune system). We have also described a new subtype, PBL arising in a chronic localized inflammatory site, without any sign of immunosuppression. At presentation, 19% of patients showed oral involvement. Immunophenotype showed CD138 positivity in 88% of cases and CD20 negativity in 90% of cases. Chemotherapy was administered to 80% of patients, with a complete response (CR) rate of 55%. The median overall survival (OS) was 32 months. In univariate analysis, HIV positive status showed better OS when compared with HIV negative status. In multivariate analysis, International Prognostic Index score, chemotherapy and CR were associated with survival benefit.
CONCLUSION(S): This cohort, the largest reported to date, increases the spectrum of knowledge on PBL, rarely described. However, specific guidelines to clarify treatment are lacking, and may improve the poor prognosis of this rare disease.
浆母细胞淋巴瘤（PBL）最初于 1997 年在 HIV 阳性患者的口腔中被描述，现在已被世界卫生组织（WHO）分类确认为一种独特的侵袭性和罕见的弥漫性大 B 细胞淋巴瘤实体。自最初描述以来，已经有其他病例报告。然而，这些病例主要来自病例报告或小系列，无法对临床特征和结局做出任何明确的结论。
报告并分析了来自法国和比利时 LYSA 中心的 135 例 PBL 患者的临床、生物学、病理学特征和结局。
中位年龄为 58 岁，男性居多。该队列分为 56 例 HIV 阳性患者、17 例移植后患者和 62 例 HIV 阴性/非移植患者。在 HIV 阴性/非移植患者中，大多数患者存在相对免疫抑制（系统性炎症性疾病、癌症史、与免疫系统减弱相关的年龄增加）。我们还描述了一种新的亚型，即起源于慢性局部炎症部位且无任何免疫抑制迹象的 PBL。在就诊时，19%的患者存在口腔受累。免疫表型显示 88%的病例 CD138 阳性，90%的病例 CD20 阴性。80%的患者接受了化疗，完全缓解（CR）率为 55%。中位总生存期（OS）为 32 个月。单因素分析显示，与 HIV 阴性状态相比，HIV 阳性状态的 OS 更好。多因素分析显示，国际预后指数评分、化疗和 CR 与生存获益相关。
该队列是迄今为止报告的最大队列，增加了对 PBL 的认识，这种疾病很少被描述。然而，缺乏明确治疗方案的具体指南，可能会改善这种罕见疾病的不良预后。
