Seliem Rania M, Griffith Rogers C, Harris Nancy L, Beheshti Javad, Schiffman Fred J, Longtine Janina, Kutok Jeffery, Ferry Judith A
*The James Homer Wright Pathology Laboratories of the Massachusetts General Hospital, Boston, MA 02114, USA.
Am J Surg Pathol. 2007 Sep;31(9):1439-45. doi: 10.1097/PAS.0b013e31804d43d8.
Human herpesvirus-8 (HHV-8) is associated with several distinct lymphoproliferative disorders: primary effusion lymphoma, multicentric Castleman disease (MCD), MCD-associated plasmablastic lymphoma and HHV-8+, Epstein-Barr virus (EBV)+ germinotropic lymphoproliferative disorder. We report the case of a human immunodeficiency virus (HIV)+ male with fever, generalized lymphadenopathy, and splenomegaly. Two peripheral lymph nodes were excised and showed features of MCD and a prominent proliferation of HHV-8+, EBV+, CD20, CD138, MUM1+, lambda dim+, Ig heavy chain plasmablasts and immunoblasts replacing some follicles. Subsequently, a splenectomy and biopsy of retroperitoneal lymph nodes were performed; the retroperitoneal and splenic hilar lymph nodes showed changes similar to those in the peripheral lymph nodes while the markedly enlarged spleen showed replacement of occasional white pulp by the HHV-8+, EBV+ large cells. The histologic features and coinfection by EBV and HHV-8 suggested a diagnosis of HHV-8+ germinotropic lymphoproliferative disorder. However, the occurrence in an HIV+ individual, the background of MCD, the widespread anatomic distribution and the aggressive clinical course tended to exclude germinotropic lymphoproliferative disorder, and to favor multifocal plasmablastic microlymphoma. The patient died shortly after surgery; postmortem examination showed progression to overt lymphoma. The marrow showed extensive hemophagocytosis, consistent with development of a hemophagocytic syndrome. This unique case has clinical features compatible with a MCD-associated plasmablastic lymphoproliferative disorder, with pathologic features intermediate between HHV-8+ plasmablastic microlymphoma, and HHV-8+ germinotropic lymphoproliferative disorder, although in contrast to both of these, in our case, light chain expression was dim and heavy chain was not detected.
人类疱疹病毒8型（HHV-8）与多种不同的淋巴增殖性疾病相关：原发性渗出性淋巴瘤、多中心Castleman病（MCD）、MCD相关的浆母细胞淋巴瘤以及HHV-8阳性、爱泼斯坦-巴尔病毒（EBV）阳性的亲毛囊性淋巴增殖性疾病。我们报告了一例人类免疫缺陷病毒（HIV）阳性男性病例，该患者有发热、全身淋巴结肿大和脾肿大。切除了两个外周淋巴结，其表现为MCD的特征，且HHV-8阳性、EBV阳性、CD20、CD138、MUM1阳性、λ轻链弱阳性、免疫球蛋白重链浆母细胞和成免疫细胞显著增殖，取代了部分滤泡。随后进行了脾切除术及腹膜后淋巴结活检；腹膜后和脾门淋巴结的变化与外周淋巴结相似，而明显肿大的脾脏显示偶尔有白髓被HHV-8阳性、EBV阳性的大细胞取代。组织学特征以及EBV和HHV-8的共感染提示诊断为HHV-8阳性亲毛囊性淋巴增殖性疾病。然而，该病例发生于HIV阳性个体、存在MCD背景、解剖学分布广泛以及临床病程侵袭性强，这些倾向于排除亲毛囊性淋巴增殖性疾病，而支持多灶性浆母细胞微淋巴瘤。患者术后不久死亡；尸检显示已进展为明显的淋巴瘤。骨髓显示广泛的噬血细胞现象，符合噬血细胞综合征的发展。这个独特的病例具有与MCD相关的浆母细胞淋巴增殖性疾病相符的临床特征，其病理特征介于HHV-8阳性浆母细胞微淋巴瘤和HHV-8阳性亲毛囊性淋巴增殖性疾病之间，不过与这两者不同的是，在我们的病例中，轻链表达弱阳性且未检测到重链。
