Centre de Compétence Maladies Pulmonaires Rares, Hôpital Bichat, APHP, Paris.
Eur Respir J. 2013 Oct;42(4):1105-18. doi: 10.1183/09031936.00154212. Epub 2013 Feb 8.
Human herpesvirus (HHV)-8 is an oncogenic gamma herpesvirus that was first described in 1994 in Kaposi sarcoma lesions. HHV-8 is involved in the pathophysiological features of multicentric Castleman's disease (MCD) and primary effusion lymphoma (PEL), both rare B-cell lymphoproliferative diseases. HHV-8-related tumours occur almost exclusively in immunocompromised patients, mostly those with HIV infection. Combined antiretroviral therapies have reduced the incidence of Kaposi sarcoma but not MCD and PEL. HHV-8-related diseases frequently exhibit pulmonary involvement, which may indicate the disease. Kaposi sarcoma in the lung is often asymptomatic but may require specific therapy. It mostly shows cutaneous or mucosal involvement. Patients with typical MCD present fever and lymphadenopathy associated with interstitial lung disease without opportunistic infection. Specific treatment may be urgent. PEL provokes a febrile, lymphocytic-exudative pleural effusion, without a pleural mass on computed tomography scan. Rapid diagnosis prevents unnecessary examinations and leads to specific, rapid treatment. Therapy is complex, combining antiretroviral therapy and chemotherapy.
人类疱疹病毒 8 型（HHV-8）是一种致瘤性γ疱疹病毒，于 1994 年在卡波西肉瘤病变中首次描述。HHV-8 参与了多中心卡斯特曼病（MCD）和原发性渗出性淋巴瘤（PEL）的病理生理特征，这两种疾病均为罕见的 B 细胞淋巴瘤。HHV-8 相关肿瘤几乎仅发生于免疫功能低下的患者，大多数是 HIV 感染者。联合抗逆转录病毒疗法降低了卡波西肉瘤的发病率，但并未降低 MCD 和 PEL 的发病率。HHV-8 相关疾病常伴有肺部受累，这可能提示疾病的存在。肺卡波西肉瘤通常无症状，但可能需要特定的治疗。它主要表现为皮肤或黏膜受累。有典型 MCD 的患者表现为发热和淋巴结病，伴有间质性肺病，但无机会性感染。可能需要紧急进行特定治疗。PEL 引起发热、淋巴细胞渗出性胸腔积液，而计算机断层扫描无胸腔肿块。快速诊断可避免不必要的检查，并可进行特定、快速的治疗。治疗较为复杂，需要联合抗逆转录病毒治疗和化疗。
