浆细胞母细胞性淋巴瘤（PBL）是一种罕见的侵袭性大细胞淋巴瘤，具有浆细胞母细胞或免疫母细胞形态学特征和终分化B细胞免疫表型。PBL常见于非淋巴结部位，常见于人类免疫缺陷病毒（HIV）和/或Epstein-Barr病毒（EBV）感染的免疫受损患者的口腔。在先前免疫功能完好的患者中，PBL单独发生于口腔外部部位的情况很少。我们报道了一例65岁的HIV-和EBV阴性男性患者，他因腹痛、疲劳和呕吐而就诊。影像学检查显示在左肾内有一个大小为30×18厘米的肿块性分叶状肿块，周围伴有腹主动脉淋巴结肿大。血清和尿液蛋白电泳显示IgA-λ型单克隆免疫球蛋白异常。肿块活检显示PBL。骨髓穿刺检查也显示了PBL的证据。患者接受化疗和放疗治疗后出现了初步改善，但在初步诊断后14个月死亡。根据我们的文献回顾，这种PBL病例是少数报道中涉及免疫功能正常、HIV-和EBV阴性患者具有肾脏肿块的病例之一。区分PBL和浆细胞骨髓瘤（PCM）可能具有挑战性。了解临床特征，包括存在CRAB（高钙血症、肾功能衰竭、贫血、骨损害）或骨髓被成熟克隆浆细胞浸润等情况，有助于确立PCM的诊断。PCM的基因特征（典型易位或突变）也有助于区分PCM的浆细胞母细胞转化和PBL。我们报道的病例还凸显了需要进行更多研究以确定特异免疫组化和分子标记物，以提高免疫功能正常患者PBL的诊断能力。 版权所有 © 2023 Elsevier Inc. 发表

Plasmablastic lymphoma (PBL) is a rare, aggressive large B-cell lymphoma with plasmablastic or immunoblastic morphology and a terminally differentiated B-cell immunophenotype. PBL often presents at extranodal sites, commonly the oral cavity of immunocompromised patients with human immunodeficiency virus (HIV) and/or Epstein-Barr virus (EBV) infection. Cases of PBL arising outside the oral cavity in previously healthy immunocompetent patients are rare.We report a 65-year-old HIV- and EBV-negative man who presented with abdominal pain, fatigue, and vomiting. Imaging studies showed a 30 × 18 cm bulky lobulated mass located within the left kidney with surrounding para-aortic lymphadenopathy. Serum and urine protein electrophoresis revealed a monoclonal gammopathy of IgA lambda type. Biopsy of the mass showed PBL. Bone marrow lumbar puncture evaluations also showed evidence of PBL. The patient was treated with chemotherapy and radiation with initial improvement; however, he died 14 months after initial diagnosis.Based on our literature review, this case of PBL is one of the few reported to present as a kidney mass in immunocompetent, HIV- and EBV-negative patient. Distinguishing PBL from plasma cell myeloma (PCM) can be challenging. Knowledge of clinical features including presence of CRAB (hypercalcemia, renal failure, anemia, bone lesions) or bone marrow infiltration by mature clonal plasma cells is helpful to establish a diagnosis of PCM. Genetic features of PCM (typical translocations or mutations) also can be helpful in distinguishing plasmablastic transformation of PCM and from PBL. The case we report also highlights the need for more studies to identify specific immunohistochemical and molecular markers to improve PBL diagnosis in immunocompetent patients.Copyright © 2023. Published by Elsevier Inc.