Suichi Tomoki, Misawa Sonoko, Sekiguchi Yukari, Shibuya Kazumoto, Tsuneyama Atsuko, Suzuki Yo-Ichi, Nakamura Keigo, Kano Hiroki, Kuwabara Satoshi
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
J Neurol Sci. 2020 Jun 15;413:116771. doi: 10.1016/j.jns.2020.116771. Epub 2020 Mar 6.
POEMS (polyneuropathy, organomegaly, endocrinopathy monoclonal gammopathy, and skin changes) syndrome is occasionally associated with Castleman disease (CD) and their prognosis is considered as poorer than that in POEMS alone patients. To elucidate recent prognosis of POEMS syndrome coexisting with CD, we reviewed clinical data of 102 patients with POEMS syndrome treated at our institution between 2000 and 2018 and compared clinical characteristics, response to treatment, and prognosis between POEMS patients with biopsy-proven CD (POEMS-CD) and those without it. Fourteen POEMS-CD patients and 56 POEMS alone patients were identified, and the remaining 32 patients with unbiopsied lymphadenopathy were excluded. POEMS-CD patients significantly showed earlier onset and less severe neuropathic symptoms. Most of the POEMS-CD patients were treated with thalidomide and dexamethasone (n = 10, 71%), and subsequently received autologous stem cell transplantation (n = 6, 43%). Response to thalidomide was better in patients with POEMS-CD than those with POEMS alone (90% vs 43% clinical response, [p = .012]; 80% vs 45% normalization of serum VEGF levels, [p = .079]). The 10-year overall survival (95% confidence interval) was 89% (50-98%) in POEMS-CD patients and 61% (42-77%) in those with POEMS alone. POEMS syndrome associated with CD constitutes a subgroup of POEMS syndromes characterized by earlier onset, mild polyneuropathy, and favorable response to treatment. Recognition of this subgroup is significant for determination of therapeutic strategy.
POEMS（多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变）综合征偶尔与Castleman病（CD）相关，其预后被认为比单纯POEMS患者更差。为了阐明与CD共存的POEMS综合征的近期预后，我们回顾了2000年至2018年在我院接受治疗的102例POEMS综合征患者的临床资料，并比较了经活检证实患有CD的POEMS患者（POEMS-CD）和未患CD的POEMS患者的临床特征、治疗反应和预后。确定了14例POEMS-CD患者和56例单纯POEMS患者，其余32例有未活检淋巴结病的患者被排除。POEMS-CD患者显著表现为起病较早且神经病变症状较轻。大多数POEMS-CD患者接受沙利度胺和地塞米松治疗（n = 10，71%），随后接受自体干细胞移植（n = 6，43%）。POEMS-CD患者对沙利度胺的反应优于单纯POEMS患者（临床反应率90%对43%，[p = 0.012]；血清VEGF水平正常化率80%对45%，[p = 0.079]）。POEMS-CD患者的10年总生存率（95%置信区间）为89%（50 - 98%），单纯POEMS患者为61%（42 - 77%）。与CD相关的POEMS综合征构成POEMS综合征的一个亚组，其特征为起病较早、多发性神经病较轻且对治疗反应良好。认识到这一亚组对于确定治疗策略具有重要意义。
