Usmani Amena, Walts Ann E, Patel Snehalkumar, Alkan Serhan, Kitahara Sumire
Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, California.
Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, California.
J Am Soc Cytopathol. 2015 Jan-Feb;4(1):37-43. doi: 10.1016/j.jasc.2014.10.002. Epub 2014 Oct 8.
Human herpesvirus 8-positive (HHV8+) primary effusion lymphoma is a well-recognized clinicopathologic entity. In contrast, HHV8-negative (HHV8-) effusion-based lymphoma (EBL) is incompletely characterized and under-recognized. We describe 17 cases of HHV8- EBL at our institution.
Cytology and available immunohistochemistry and cytogenetics were reviewed. Patient demographics, history, and outcome were obtained from medical records.
The effusions were pleural (n = 9; 53%), peritoneal (n = 4; 24%), pericardial (n = 3; 18%), and pleural and pericardial (n = 1; 6%). Fifteen cases (88%) were CD20+ and 15 had sufficient information for classification by Hans algorithm (CD10, BCL6, MUM1): 11 (73%) nongerminal center and 4 (27%) germinal center phenotype. Epstein-Barr virus in situ hybridization was negative in 16 cases (94%). Three of 14 cases were MYC+ by immunohistochemistry. Fluorescence in situ hybridization, performed on 5 of the 17 cases, showed a MYC rearrangement in 1 case and a BCL6 rearrangement in 2 cases. Most patients were elderly (median age 86 years) and female (82%). Human immunodeficiency virus testing results, available in 4 patients, were negative. Seven (41%), including 1 of 2 heart transplant recipients, had congestive heart failure. Follow-up (5 days to 12 years) was available for 16 patients including 4 who survived ≥8 years. Only 1 of the 8 known deaths was clinically attributed to lymphoma.
HHV8- lymphomas that occur in body cavity effusions without detectable lymphomatous masses are usually composed of large CD20+ lymphoid or lymphoplasmacytoid cells. In contrast to HHV8+ primary effusion lymphoma, patients with HHV8- EBL are usually elderly, lack a documented human immunodeficiency virus-positive history, and have a longer disease specific survival.
人疱疹病毒8阳性（HHV8+）原发性渗出性淋巴瘤是一种公认的临床病理实体。相比之下，HHV8阴性（HHV8-）渗出性淋巴瘤（EBL）的特征尚未完全明确，且未得到充分认识。我们描述了本机构收治的17例HHV8-EBL病例。
回顾了细胞学以及可用的免疫组织化学和细胞遗传学检查结果。从病历中获取患者的人口统计学资料、病史和预后情况。
渗出液分别为胸腔积液（n = 9；53%）、腹腔积液（n = 4；24%）、心包积液（n = 3；18%）以及胸腔和心包积液（n = 1；6%）。15例（88%）为CD20阳性，其中15例有足够信息可通过汉斯算法（CD10、BCL6、MUM1）进行分类：11例（73%）为非生发中心型，4例（27%）为生发中心型。16例（94%）的爱泼斯坦-巴尔病毒原位杂交结果为阴性。14例中有3例免疫组织化学检测显示MYC阳性。对17例中的5例进行荧光原位杂交检测，结果显示1例存在MYC重排，2例存在BCL6重排。大多数患者为老年人（中位年龄86岁），且为女性（82%）。4例患者的人类免疫缺陷病毒检测结果为阴性。7例（41%）患者患有充血性心力衰竭，其中包括2例心脏移植受者中的1例。16例患者有随访记录（5天至12年），其中4例存活时间≥8年。已知的8例死亡病例中，只有1例临床诊断为死于淋巴瘤。
发生于体腔积液且未发现淋巴瘤肿块的HHV8-淋巴瘤通常由大的CD20阳性淋巴细胞或淋巴浆细胞样细胞组成。与HHV8+原发性渗出性淋巴瘤不同，HHV8-EBL患者通常为老年人，无人类免疫缺陷病毒阳性病史记录，且疾病特异性生存期较长。
