Sorbonne Université, AP-HP, Hôpital Tenon, Service de médecine interne, Centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (CEREMAIA) , Paris , France.
Clinical Immunology Department, National Reference Center for Castleman Disease and UMR 1149 CRI INSERM, Hôpital Saint Louis, Assistance Publique Hôpitaux de Paris (APHP) Université Paris Diderot , Paris , France.
Amyloid. 2019 Dec;26(4):197-202. doi: 10.1080/13506129.2019.1641078. Epub 2019 Jul 31.
HHV8-negative Castleman disease (CD) is classified as hyaline vascular (HV) type, or mixed or plasma cell (PC) types. It may present as multicentric CD (MCD) or unicentric CD (UCD). CD is a rare cause of AA amyloidosis (AAA). We aimed to report the main features of CD with secondary AAA through a description of new cases and a systematic literature review. New cases were identified from the French National Reference Center for AAA. A systematic literature review was performed to identify HHV8-negative CD cases associated with AAA. Thirty-seven patients were analysed, consisting of two new cases and 35 from literature. Twenty-three had UCD and 14 had MCD. PC was the main histologic subtype ( = 25; 68%) in both UCD and MCD patients. Surgical excision of UCD was performed in 21 patients (91%) with a favourable outcome, except for four patients (19%). Clinical and biologic remission was achieved in six patients with MCD (43%), all of whom were treated with anti-interleukin-6 (IL-6) therapy. AAA is a rare complication of CD, namely idiopathic MCD and UCD presenting with the PC histologic subtype. Surgical excision of UCD should be the first-line treatment whenever possible, while anti-IL-6 therapies seem effective for MCD.
HHV8 阴性卡斯特曼病（CD）分为透明血管（HV）型或混合或浆细胞（PC）型。它可能表现为多中心 CD（MCD）或单中心 CD（UCD）。CD 是 AA 淀粉样变性（AAA）的罕见原因。我们旨在通过描述新病例和系统文献复习，报告继发于 AAA 的 CD 的主要特征。 新病例是从法国国家 AAA 参考中心确定的。进行了系统的文献复习，以确定与 AAA 相关的 HHV8 阴性 CD 病例。 分析了 37 例患者，包括 2 例新病例和 35 例文献病例。23 例为 UCD，14 例为 MCD。UCD 和 MCD 患者的主要组织学亚型均为 PC（分别为 25 例；68%）。21 例 UCD 患者（91%）行手术切除，除 4 例（19%）外，均取得良好疗效。6 例 MCD 患者（43%）临床和生物学缓解，均接受抗白细胞介素 6（IL-6）治疗。 AAA 是 CD 的罕见并发症，即特发性 MCD 和 UCD 伴 PC 组织学亚型。只要可能，UCD 的手术切除应作为一线治疗，而抗 IL-6 治疗似乎对 MCD 有效。
