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Abstract：
Primary effusion lymphoma (PEL) is a very rare non-Hodgkin lymphoma caused by human herpesvirus-8 (HHV8) that grows in liquid phase within body cavities. The diagnosis of PEL is based on cytology but requires confirmatory ancillary tests. PEL occurs mainly in association with HIV infection. This study describes 9 cases of PEL in HIV-negative patients and compares their characteristics with 10 HIV-associated cases of PEL diagnosed at a single institution in Italy between 1995 and 2019.
Clinical records were reviewed for demographic data, comorbidities, laboratory abnormalities, and outcome. PEL samples were evaluated for cytomorphology, immunophenotype, immunoglobulin (IG)/T cell receptor (TR) rearrangements, and HHV8 and Epstein-Barr virus (EBV) viral loads in effusion supernatants.
HIV-unrelated PEL occurred in 8 elderly patients (7 men, 1 woman) and 1 young adult with primary antibody deficiency. Cytology revealed HHV8-positive lymphoma cells lacking B/T cell antigens and exhibiting 2 cell patterns (polymorphous or monotonous). IG was clonally rearranged in all cases; aberrant TRG occurred in 2 cases. Effusion supernatants had more than 106 HHV8 DNA copies per mL and variable loads of EBV DNA. Compared with HIV-associated PEL, the HIV-negative cohort was characterized by older age, less frequent association with Kaposi sarcoma and/or multicentric Castleman disease, comparable but less abnormal laboratory parameters, and a nonsignificant survival benefit. PEL cases with low apoptosis were associated with better prognosis.
To the best of our knowledge, our case series of HIV-unrelated PEL is the largest thus far, expands the spectrum of cytological findings, and supports the need for a multidisciplinary approach in the diagnostic workup.
Clinical records were reviewed for demographic data, comorbidities, laboratory abnormalities, and outcome. PEL samples were evaluated for cytomorphology, immunophenotype, immunoglobulin (IG)/T cell receptor (TR) rearrangements, and HHV8 and Epstein-Barr virus (EBV) viral loads in effusion supernatants.
HIV-unrelated PEL occurred in 8 elderly patients (7 men, 1 woman) and 1 young adult with primary antibody deficiency. Cytology revealed HHV8-positive lymphoma cells lacking B/T cell antigens and exhibiting 2 cell patterns (polymorphous or monotonous). IG was clonally rearranged in all cases; aberrant TRG occurred in 2 cases. Effusion supernatants had more than 106 HHV8 DNA copies per mL and variable loads of EBV DNA. Compared with HIV-associated PEL, the HIV-negative cohort was characterized by older age, less frequent association with Kaposi sarcoma and/or multicentric Castleman disease, comparable but less abnormal laboratory parameters, and a nonsignificant survival benefit. PEL cases with low apoptosis were associated with better prognosis.
To the best of our knowledge, our case series of HIV-unrelated PEL is the largest thus far, expands the spectrum of cytological findings, and supports the need for a multidisciplinary approach in the diagnostic workup.
摘要：
原发性渗出性淋巴瘤（PEL）是由人疱疹病毒8（HHV8）引起的非常罕见的非霍奇金淋巴瘤，在体腔内以液相生长。PEL的诊断基于细胞学，但需要确证的辅助测试。PEL主要与HIV感染有关。这项研究描述了9例HIV阴性患者的PEL病例，并将其特征与1995年至2019年间在意大利一家机构诊断的10例HIV相关PEL病例进行了比较。
对临床记录进行了人口统计学数据审查，合并症,实验室异常，和结果。对PEL样本进行了细胞形态学评估，免疫表型,免疫球蛋白（IG）/T细胞受体（TR）重排，和HHV8和EB病毒(EBV)在积液上清液中的病毒载量。
8名老年患者（7名男性，1名女性）和1名初级抗体缺乏的年轻成年人。细胞学显示HHV8阳性淋巴瘤细胞缺乏B/T细胞抗原，并表现出2种细胞模式（多形性或单调性）。所有病例均有IG克隆重排；2例发生异常TRG。流出物上清液具有超过106个HHV8DNA拷贝/mL和可变的EBVDNA载量。与HIV相关的PEL相比，HIV阴性队列的特征是年龄较大，与卡波西肉瘤和/或多中心Castleman病的相关性较低，可比较但不太异常的实验室参数，和不显著的生存益处。低细胞凋亡的PEL病例预后较好。
据我们所知,我们的艾滋病毒无关的PEL病例系列是迄今为止最大的，扩大了细胞学发现的范围，并支持在诊断检查中需要多学科方法。
对临床记录进行了人口统计学数据审查，合并症,实验室异常，和结果。对PEL样本进行了细胞形态学评估，免疫表型,免疫球蛋白（IG）/T细胞受体（TR）重排，和HHV8和EB病毒(EBV)在积液上清液中的病毒载量。
8名老年患者（7名男性，1名女性）和1名初级抗体缺乏的年轻成年人。细胞学显示HHV8阳性淋巴瘤细胞缺乏B/T细胞抗原，并表现出2种细胞模式（多形性或单调性）。所有病例均有IG克隆重排；2例发生异常TRG。流出物上清液具有超过106个HHV8DNA拷贝/mL和可变的EBVDNA载量。与HIV相关的PEL相比，HIV阴性队列的特征是年龄较大，与卡波西肉瘤和/或多中心Castleman病的相关性较低，可比较但不太异常的实验室参数，和不显著的生存益处。低细胞凋亡的PEL病例预后较好。
据我们所知,我们的艾滋病毒无关的PEL病例系列是迄今为止最大的，扩大了细胞学发现的范围，并支持在诊断检查中需要多学科方法。
