Abid Muhammad Bilal, Peck Rachel, Abid Muhammad Abbas, Al-Sakkaf Wesam, Zhang Yuening, Dunnill Giles S, Staines Konrad, Sequeiros Iara-Maria, Lowry Lisa
Department of Haematology, University Hospitals of Bristol NHS Trust, Bristol, UK.
Division of Otolaryngology and Head and Neck Surgery, John Hopkins School of Medicine, Baltimore, MD, USA.
Hematol Oncol. 2018 Feb;36(1):320-323. doi: 10.1002/hon.2420. Epub 2017 Apr 11.
Castleman disease is a rare lymphoproliferative disorder with 2 distinctly defined clinical forms. While multicentric Castleman disease (UCD) poses a potential therapeutic challenge, unicentric variant has historically been considered curable with surgical resection. Hence, little is known to guide management of patients with UCD, refractory to surgical resection and combination chemotherapy. We present a case of a patient, negative for HIV and HHV-8, who had an unsuccessful surgical intervention and no response to radiotherapy and chemotherapy. He had severe paraneoplastic pemphigus and was treated with tocilizumab, an anti-interleukin-6 receptor monoclonal antibody that has demonstrated good response rates in multicentric Castleman disease but demonstrated no clinical response despite 2 months of treatment. Our report is the first to describe a lack of response to tocilizumab in the rare setting of refractory UCD and discuss potential for distinct disease biology.
Castleman病是一种罕见的淋巴增生性疾病，有两种明确界定的临床类型。虽然多中心Castleman病（UCD）带来了潜在的治疗挑战，但单中心型以往被认为手术切除可治愈。因此，对于手术切除和联合化疗难治的UCD患者的管理，了解甚少。我们报告一例患者，HIV和HHV - 8检测均为阴性，手术干预未成功，放疗和化疗均无反应。他患有严重的副肿瘤性天疱疮，接受了托珠单抗治疗，托珠单抗是一种抗白细胞介素 - 6受体单克隆抗体，在多中心Castleman病中显示出良好的反应率，但尽管治疗了2个月仍无临床反应。我们的报告首次描述了在难治性UCD这种罕见情况下对托珠单抗无反应，并讨论了不同疾病生物学的可能性。
