Darwish Ilyse, Costiniuk Cecilia, Kronfli Nadine, Haegert David, Routy Jean-Pierre
Division of Infectious Diseases, McGill University Health Centre, Montréal, Quebec, Canada.
Chronic Viral Illness Service, McGill University Health Centre, Montréal, Quebec, Canada.
IDCases. 2021 Oct 20;26:e01315. doi: 10.1016/j.idcr.2021.e01315. eCollection 2021.
Human herpesvirus-8 (HHV8)-associated multicentric Castleman disease (HHV8-MCD) is a rare nonmalignant lymphoproliferative disorder most commonly observed in PLWH. Herein, we describe an HIV-infected adult male from Cameroon with relapsing HHV8-MCD (HIV+MCD). The patient developed constitutional symptoms, diffuse lymphadenopathy, thrombocytopenia and autoimmune hemolytic anemia. Excisional lymph node biopsy findings were consistent with HHV8-MCD. He was successfully treated with corticosteroids and rituximab. One year later, he developed relapsing disease and was successfully treated again with rituximab. Interestingly, HIV viral load blips correlate with MCD flares, suggesting that low-level viremia is linked with T-cell clonal expansion and/or inflammation, rather than a lack of effective antiretroviral therapy. Rituximab either alone or in combination with chemotherapy for aggressive disease is the standard of care, with approximately 95% of treated patients achieving complete remission. Despite highly effective therapy, HIV+MCD often presents with a relapsing and remitting disease course and carries an increased risk for the development of HHV8-associated lymphoma.
人类疱疹病毒8型(HHV8)相关的多中心Castleman病(HHV8-MCD)是一种罕见的非恶性淋巴增殖性疾病,最常见于艾滋病毒感染者(PLWH)。在此,我们描述了一名来自喀麦隆的感染艾滋病毒的成年男性,患有复发性HHV8-MCD(HIV+MCD)。该患者出现全身症状、弥漫性淋巴结病、血小板减少和自身免疫性溶血性贫血。切除性淋巴结活检结果与HHV8-MCD一致。他接受皮质类固醇和利妥昔单抗治疗后获得成功。一年后,他疾病复发,再次接受利妥昔单抗治疗成功。有趣的是,艾滋病毒病毒载量波动与MCD发作相关,这表明低水平病毒血症与T细胞克隆扩增和/或炎症有关,而不是缺乏有效的抗逆转录病毒治疗。利妥昔单抗单独使用或与化疗联合用于侵袭性疾病是标准治疗方法,约95%的接受治疗的患者实现完全缓解。尽管有高效治疗,但HIV+MCD通常表现为复发和缓解的病程,并且发生HHV8相关淋巴瘤的风险增加。