Tinguely M, Vonlanthen R, Müller E, Dommann-Scherrer C C, Schneider J, Laissue J A, Borisch B
Institute of Pathology, University of Berne, Switzerland.
Mod Pathol. 1998 Apr;11(4):307-12.
Epstein-Barr virus (EBV)-associated lymphoproliferative diseases are a frequent occurrence in immunodeficient patients. Most commonly seen are polymorphic B-cell lymphoproliferative disorders and malignant B-cell lymphomas. Classical Hodgkin's disease (HD), however, is rare in these patients. In the present study, we attempted to characterize cases resembling HD in patients with a variety of underlying immunodeficiency states using clinical aspects, immunohistochemistry, in situ hybridization, and polymerase chain reaction. All of the six cases that we investigated presented clinically with B symptoms and a short, devastating course. Histologically, they showed a lymphocytic depletion and multiple cells that resembled HD and Reed-Sternberg cells. Many of those were atypical blast cells that stained positively for B-cell surface antigens, such as CD20 and CD79a, whereas others were of the typical HD and Reed-Sternberg phenotype. Another frequent finding, especially in the extranodal sites, was a perivascular arrangement of large blast cells intermingled with small lymphoid cells. All of the cases were EBV-associated (proved latent membrane protein-1 immunohistochemical analysis, EBV-encoded RNA in situ hybridization, and polymerase chain reaction for subtypes A and B), with a frequent coinfection of type A and B. This was in contrast to ordinary HD, which is characterized by single infection of only one strain, i.e., the subtype A in Western countries. Three cases showed clonal B-cell populations, at least at terminal stages of the disease. We describe a lymphoproliferative disorder in immunodeficient patients with features of HD that, in some respects, resembles an EBV-driven lymphoproliferative disorder seen in cases of fatal infectious mononucleosis. We conclude that clinical and pathologic features of these disorders might cause some problems for histologic differential diagnosis and might represent a separate entity of their own in immunodeficient patients.
爱泼斯坦-巴尔病毒（EBV）相关的淋巴增殖性疾病在免疫缺陷患者中很常见。最常见的是多形性B细胞淋巴增殖性疾病和恶性B细胞淋巴瘤。然而，经典霍奇金淋巴瘤（HD）在这些患者中很少见。在本研究中，我们试图通过临床症状、免疫组织化学、原位杂交和聚合酶链反应，对患有各种潜在免疫缺陷状态的患者中类似HD的病例进行特征描述。我们研究的所有6例病例临床上均表现为B症状且病程短、病情严重。组织学上，它们表现为淋巴细胞耗竭以及多个类似HD和里德-斯腾伯格细胞的细胞。其中许多是对B细胞表面抗原（如CD20和CD79a）呈阳性染色的非典型母细胞，而其他细胞则具有典型的HD和里德-斯腾伯格表型。另一个常见发现，尤其是在结外部位，是大母细胞与小淋巴细胞混合的血管周围排列。所有病例均与EBV相关（通过潜伏膜蛋白-1免疫组织化学分析、EBV编码RNA原位杂交以及A和B亚型的聚合酶链反应证实），且常伴有A和B型的混合感染。这与普通HD不同，普通HD的特征是仅感染一种毒株，即西方国家的A亚型。3例病例至少在疾病终末期显示出克隆性B细胞群体。我们描述了一种免疫缺陷患者中具有HD特征的淋巴增殖性疾病，在某些方面类似于在致命传染性单核细胞增多症病例中所见的EBV驱动的淋巴增殖性疾病。我们得出结论，这些疾病的临床和病理特征可能会给组织学鉴别诊断带来一些问题，并且可能代表免疫缺陷患者中一种独立的实体。
