年龄相关的EBV-B细胞淋巴增殖性疾病

Age-related Epstein-Barr virus-associated B-cell lymphoproliferative disorders:Special references to lymphomas surrounding this newly recognized clinicopathologic disease

EB病毒(EBV)与某些恶性淋巴瘤相关,包括伯基特(Burkitt)淋巴瘤、霍奇金淋巴瘤(HL)、免疫缺陷相关的淋巴增殖性疾患(LPD)和部分弥漫性大B细胞淋巴瘤亚组。Shimoyama等最近确认系列无免疫缺陷的老年EBV相关(EBV+)B细胞LPD(B-LPD),在许多方面与免疫缺陷相关的LPD相类似,特称该类新生物为"老年性"("senile")或年龄相关的EBV+B-LPDs。EBV+B-LPD与弥漫性大B细胞淋巴瘤患者随着年龄增加其相对比例较高,≥90岁达到发病高峰(20%-30%),中位年龄71岁,可能与随着年龄的老化所发生的免疫学恶化有关。该病的病理学特征是具有中心母细胞、免疫母细胞、霍奇金和Reed-Sternberg(HRS)样巨细胞及伴不同程度的反应性细胞成分,是血液学家和病理学家面临的鉴别诊断和治疗选择问题。本文进一步阐明该种新认识的年龄相关的EBV+B-LPD和EBV+HL包括形态学和生物学在内的临床病理学特点。

Epstein - Barr virus （EBV） is associated with some disease entities of malignant lymphomas, including Burkitt lymphoma, Hodgkin lymphoma, immunodeficiency - associated lymphoproliferative disorders （ LPD ）, and a part of diffuse large B- cell lymphoma. Studies have recently identified a series of elderly patients with EBV-associated （or EBV＋ ） B - cell LPD （ B - LPD） showing similarities in many respects to immunodeficiency - associated LPD, although no evidence of underlying immunodeficiency was found. Therefore, the nosological category of senile or age - related EBV ＋ B - LPD has been proposed for those patients. A larger series of patients with this disease revealed that the relative ratios of such EBV ＋ B - LPD to all diffuse large B - cell lymphoma cases were higher with increasing age, reaching a peak （20% -30% ） at ≥90 years of age, with a median of 71 years, providing additional evidence for this disease may be related to immunological deterioration as a result of the aging process. This new disease entity is characterized pathologically by centmblasts, immunoblasts, and Hodgkin and Reed - Sternberglike giant cells with a varying degree of reactive components, often posing therapeutic and diagnostic problems for hematologists and pathologists, respectively. The aim of the present review is to briefly summarize the overall clinicopathological profile of this newly recognized age - related （ also called senile＇） EBV＋ B - LPD and EBV ＋ Hodgkin lymphoma for a practical diagnostic approach.