多中心HHV8阳性Castleman病病程中白塞病的复发：化疗/放疗联合α干扰素治疗方案实现长期完全缓解

Reactivation of Behçet's disease in the course of multicentric HHV8-positive Castleman's disease: long-term complete remission by a combined chemo/radiation and interferon-alpha therapy regimen.

作者信息

Strohal R, Tschachler E, Breyer S, Uthman A, Simonitsch I, Tratting S, Scheithauer W, Stingl G, Kornek G V

机构信息

Department of Dermatology, University of Vienna School of Medicine, Austria.

出版信息

Br J Haematol. 1998 Dec;103(3):788-90. doi: 10.1046/j.1365-2141.1998.01038.x.

DOI:10.1046/j.1365-2141.1998.01038.x

PMID:9858232

Abstract

We used a new combined chemo- (COP/ABVD), radiation and interferon-alpha (10 x 10(6) IU s.c. 3x per week/12 months) therapy regimen to treat severe multicentric Castleman's disease (CD) complicated by relapsing Behcet's disease (BD) manifestations. More than 16 years after diagnosis of CD the patient remains in very good clinical condition, with remission of all CD and BD manifestations 13 months after discontinuation of the interferon-alpha treatment. In addition, our clinicopathological, immunohistological and virological data suggest a pathogenetic link between CD and BD via activation of pre-existing BD-specific plasma cells due to CD-related HHV8-induced overexpression of interleukin-6.

摘要

我们采用了一种新的联合化疗（COP/ABVD）、放疗及α-干扰素（10×10⁶国际单位，皮下注射，每周3次，共12个月）治疗方案，用于治疗合并复发性白塞病（BD）表现的重度多中心Castleman病（CD）。在诊断CD超过16年后，患者临床状况依然良好，在停用α-干扰素治疗13个月后，所有CD和BD表现均缓解。此外，我们的临床病理、免疫组织化学及病毒学数据表明，由于CD相关的人疱疹病毒8（HHV8）诱导白细胞介素-6过度表达，激活了已存在的BD特异性浆细胞，从而提示CD与BD之间存在发病机制上的联系。