Gonzalez-Farre Blanca, Martinez Daniel, Lopez-Guerra Monica, Xipell Marc, Monclus Ester, Rovira Jordina, Garcia Felipe, Lopez-Guillermo Armando, Colomo Luis, Campo Elias, Martinez Antonio
Department of Pathology, Hospital Clinic, Institut d'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Department of Geriatrics, Hospital Clinic, University of Barcelona, Barcelona, Spain.
Mod Pathol. 2017 May;30(5):745-760. doi: 10.1038/modpathol.2016.233. Epub 2017 Jan 13.
Human herpesvirus 8 (HHV8)-associated lymphoid proliferations are uncommon and poorly characterized disorders mainly affecting immunosuppressed patients, especially with HIV infection. They encompass different diseases with overlapping features that complicate their classification. In addition, the role of HHV8 in reactive lymphoid hyperplasia is not well known. To analyze the clinicopathological spectrum of these lesions, we have reviewed 66 biopsies of 61 patients with HHV8 infection. All cases were also investigated for Epstein-Barr virus (EBV) and HIV infection. We identified 13 (20%) cases of HHV8-related reactive lymphoid hyperplasia, 2 (3%) HHV8 plasmablastic proliferations of the splenic red pulp, 28 (42%) multicentric Castleman disease, 6 (9%) germinotropic lymphoproliferative disorders, and 17 (26%) HHV8-related lymphomas. As expected, the pathologic subtype was predictive of overall survival (P<0.05). Forty-seven of our cases were HIV positive (77%). In addition to the classical presentation of the different entities, we identified novel and overlapping features. Reactive HHV8 proliferations were frequently associated with systemic symptoms but never progressed to overt HHV8-positive lymphoma. Two cases had a plasmablastic proliferation limited to spleen. Eight cases of multicentric Castleman disease had a previously unrecognized presentation shortly after the diagnosis of HIV infection, six cases had cavity effusions, and three showed plasmablast enriched proliferations. One germinotropic lymphoproliferative disorder was EBV negative and three occurred in HIV-positive patients, who had distinctive clinical and morphological features. Two of the HHV8-related lymphomas did not fulfill the criteria for previously recognized entities. All these findings expand the clinical and pathological spectrum of HHV8-related lymphoid proliferations, which is broader than current recognized.
人疱疹病毒8型（HHV8）相关的淋巴组织增生是一类罕见且特征不明的疾病，主要影响免疫抑制患者，尤其是合并HIV感染的患者。它们包括具有重叠特征的不同疾病，这使得它们的分类变得复杂。此外，HHV8在反应性淋巴组织增生中的作用尚不清楚。为了分析这些病变的临床病理谱，我们回顾了61例HHV8感染患者的66份活检标本。所有病例还检测了EB病毒（EBV）和HIV感染情况。我们识别出13例（20%）HHV8相关的反应性淋巴组织增生、2例（3%）脾红髓的HHV8浆母细胞增生、28例（42%）多中心Castleman病、6例（9%）亲嗜性淋巴细胞增生性疾病以及17例（26%）HHV8相关淋巴瘤。正如预期的那样，病理亚型可预测总生存期（P<0.05）。我们的病例中有47例HIV阳性（77%）。除了不同实体的典型表现外，我们还识别出了新的和重叠的特征。反应性HHV8增生常伴有全身症状，但从未进展为明显的HHV8阳性淋巴瘤。2例患者的浆母细胞增生仅限于脾脏。8例多中心Castleman病患者在诊断HIV感染后不久出现了先前未被认识的表现，6例有胸腔积液，3例表现为富含浆母细胞的增生。1例亲嗜性淋巴细胞增生性疾病EBV阴性，3例发生在HIV阳性患者中，这些患者具有独特的临床和形态学特征。2例HHV8相关淋巴瘤不符合先前公认实体的标准。所有这些发现扩展了HHV8相关淋巴组织增生的临床和病理谱，其比目前所认识的更为广泛。
