Treatment and outcome of Unicentric Castleman Disease: a retrospective analysis of 71 cases

Treatment and outcome of Unicentric Castleman Disease: a retrospective analysis of 71 cases
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DOI:
10.1111/bjh.15921
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发表时间:
2019-07-01
影响因子:
6.5
通讯作者:
Galicier, Lionel
Galicier, Lionel
中科院分区:
医学2区
文献类型:
--
作者:
Boutboul, David;Fadlallah, Jehane;Galicier, Lionel

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我们回顾性分析了71例单中心性Castleman病,这是一种罕见的,通常无症状的良性淋巴增生性疾病,表现为独特的淋巴结肿块。虽然手术被认为是金标准治疗,但只有38例(54%)患者接受了最初的手术切除,95%的患者治愈。另有9名患者在尝试医疗复位后进行了手术。21例患者采用减量治疗,有效率为55%,但没有最佳方案的证据。由于相关毒性,放疗仅限于8例患者。在13名无症状患者中考虑观察和等待,其中11名患者保持稳定长达17年。
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.