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.
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。