Wadsworth Paul A, Miranda Roberto N, Bhakta Pooja, Bhargava Peeyush, Weaver Dylan, Dong Jianli, Ovechko Vasily, Norman Michael, Muthukumarana Palawinnage V, Bayes Mathew G, Mallick Jayati, Lyapichev Kirill A
Department of Pathology The University of Texas Medical Branch Galveston Texas USA.
Department of Pathology Stanford University Stanford California USA.
EJHaem. 2023 Jan 24;4(1):226-231. doi: 10.1002/jha2.642. eCollection 2023 Feb.
Diffuse large B-cell lymphoma (DLBCL) arising in the spleen, also known as primary splenic DLBCL (PS-DLBCL), is a rare form of malignant lymphoma. It is defined as a lymphoma confined to the spleen or involving splenic hilar lymph nodes. Here we report a case of PS-DLBCL with CD30. The patient was a 62-year-old who presented with 2 weeks of left flank pain, chills, and abdominal distension. Computed tomography identified an 8-cm splenic mass with central necrosis interpreted as an abscess. A drain was placed, yielding purulent necrotic material; cytologically, only neutrophils were identified. However, purulent drainage continued for 28 days without resolution, prompting splenectomy. Pathological dissection revealed a multinodular mass with central necrosis. Microscopic examination revealed extensive karyorrhexis, abundant ghosts of large cells, and scattered large cells with pleomorphic, multilobated, and vesicular nuclei with moderately abundant cytoplasm. Immunohistochemical staining revealed large, atypical cells positive for CD20, CD30, CD45, PAX5, MYC (>40%), MUM1 (>30%), and p53 (focally). The large cells were negative for CD3 (polyclonal), CD4, CD5, CD8, CD10, CD15, CD34, BCL2, BCL6, AE1/AE3, S100, HHV8, and ALK. The Ki-67 proliferation rate was approximately 80% in large cells. Notably, this PS-DLBCL was positive for CD30, an unusual finding among non-Hodgkin B-cell lymphomas, which, coupled with the Reed-Sternberg-like morphology, raised the possibility of classic Hodgkin lymphoma. Therefore, we reviewed the literature to confirm the unique features of this large B-cell lymphoma, its abscess-like appearance, and its expression of CD30.
脾脏弥漫性大B细胞淋巴瘤(DLBCL),也称为原发性脾脏DLBCL(PS-DLBCL),是一种罕见的恶性淋巴瘤形式。它被定义为局限于脾脏或累及脾门淋巴结的淋巴瘤。在此,我们报告一例伴有CD30表达的PS-DLBCL病例。患者为一名62岁男性,出现左侧胁腹疼痛、寒战和腹胀2周。计算机断层扫描发现脾脏有一个8厘米的肿块,伴有中央坏死,考虑为脓肿。放置引流管后引出脓性坏死物质;细胞学检查仅发现中性粒细胞。然而,脓性引流持续28天未缓解,遂行脾切除术。病理检查显示为一个多结节性肿块,伴有中央坏死。显微镜检查发现广泛的核碎裂、大量大细胞的残影以及散在的大细胞,这些大细胞具有多形性、分叶状和泡状核,胞质中等丰富。免疫组化染色显示大的非典型细胞CD20、CD30、CD45、PAX5、MYC(>40%)、MUM1(>30%)和p53(局灶性)呈阳性。大细胞CD3(多克隆)、CD4、CD5、CD8、CD10、CD15、CD34、BCL2、BCL6、AE1/AE3、S100、HHV8和ALK呈阴性。大细胞中Ki-67增殖率约为80%。值得注意的是,该PS-DLBCL CD30呈阳性,这在非霍奇金B细胞淋巴瘤中是不常见的发现,再加上其里德-斯特恩伯格样形态,增加了经典霍奇金淋巴瘤的可能性。因此,我们查阅文献以确认这种大B细胞淋巴瘤的独特特征、其脓肿样外观及其CD30表达情况。