Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), Paris, France.
National Reference Centre for Castleman Disease (CRMdC), Paris, France.
Br J Haematol. 2019 Jul;186(2):269-273. doi: 10.1111/bjh.15921. Epub 2019 Apr 23.
We retrospectively analysed 71 cases of Unicentric Castleman disease, a rare, usually asymptomatic, benign lymphoproliferative disorder presenting as a unique nodal mass. Although surgery is considered as the gold standard therapy, only 38 patients (54%) underwent initial surgical resection and 95% were cured. An additional 9 patients had surgery after an attempt at medical reduction. Reduction therapy was used in 21 patients with a 55% response rate, but without evidence for an optimal regimen. Radiotherapy was limited to 8 patients because of associated toxicity. Watch and wait was considered in 13 asymptomatic patients and 11 of these remained stable for up to 17 years.
我们回顾性分析了 71 例单中心Castleman 病，这是一种罕见的、通常无症状的良性淋巴组织增生性疾病，表现为独特的淋巴结肿块。虽然手术被认为是金标准治疗方法，但只有 38 例患者（54%）接受了初始手术切除，95%的患者被治愈。另外 9 例患者在尝试药物治疗后进行了手术。21 例患者接受了减瘤治疗，反应率为 55%，但没有证据表明最佳治疗方案。由于相关毒性，放射治疗仅限于 8 例患者。13 例无症状患者采用观察等待策略，其中 11 例患者在长达 17 年的时间内保持稳定。
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