PDF(Sci-hub)
Abstract：
Pure red cell aplasia (PRCA) is a rare paraneoplastic syndrome observed in 2-5 % of thymomas. Literature reports great variability in its management. Based on an illustrative clinical case, we present a systematic literature review whose main objective is to evaluate the therapeutic management of PRCA. The literature search was performed based on the PICO method in the Medline and Scopus databases. The reference clinical case concerns a 51-year-old woman with stage IVa thymoma. After initial response to chemotherapy, a locoregional progression occurred with PRCA development that responded favorably under second line chemotherapy. The patient finally died in a context of bicytopenia with febrile neutropenia. The systematic review covers 135 articles published between 1950 and 2019. Thymectomy alone or in combination with other therapies showed a 31 % complete remission (CR) rate for PRCA of, whereas none was reported with anti-tumor treatments without thymectomy. Among immunomodulatory therapies, cyclosporin gave the highest percentage of CR (74 %). Finally, the combination of thymectomy and immunomodulatory treatments showed a CR rate of 45 %. Thymectomy appeared to be the most effective anti-tumor treatment for PRCA. Immunomodulatory therapies, particularly cyclosporine, are shown effective, but the risk of infectious complications must be considered. The optimal place of anti-tumor and immunomodulatory therapies against PRCA has yet to be determined.
摘要：
纯红细胞发育不全（PRCA）是一种罕见的副肿瘤综合征，在2-5％的胸腺瘤中观察到。文献报道其管理存在很大差异。根据一个说明性的临床案例，我们进行了系统的文献综述,其主要目的是评估PRCA的治疗管理.文献检索基于Medline和Scopus数据库中的PICO方法进行。参考临床病例涉及一名患有IVa胸腺瘤的51岁女性。在对化疗有初始反应后，PRCA发生局部进展,在二线化疗下反应良好.患者最终死于伴有发热性中性粒细胞减少的双红细胞减少症。该系统综述涵盖了1950年至2019年之间发表的135篇文章。单独或与其他疗法联合使用的胸腺切除术显示PRCA的完全缓解（CR）率为31％，而没有报道没有胸腺切除术的抗肿瘤治疗。在免疫调节疗法中，环孢菌素的CR百分比最高(74%)。最后,胸腺切除术和免疫调节治疗的组合显示CR率为45％。胸腺切除术似乎是PRCA最有效的抗肿瘤治疗方法。免疫调节疗法,特别是环孢菌素，被证明是有效的，但必须考虑感染并发症的风险。针对PRCA的抗肿瘤和免疫调节疗法的最佳位置尚未确定。
