Tuberculosis in Patients with Primary Myelofibrosis During Ruxolitinib Therapy: Case Series and Literature Review.
Tuberculosis in Patients with Primary Myelofibrosis During Ruxolitinib Therapy: Case Series and Literature Review.
复制标题
鲁索替尼治疗期间原发性骨髓纤维化患者的结核病：病例系列和文献综述
DOI：
10.2147/idr.s267997
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发表时间：
2020
影响因子：
3.9
通讯作者：
Hong Z
中科院分区：
文献类型：
--
作者：
Peng Y;Meng L;Hu X;Han Z;Hong Z
背景：选择性Janus激活的激酶抑制剂鲁索利替尼(ruxolitinib，Rux)具有明显的减轻脾肿大和改善机体症状的作用，目前广泛用于治疗骨髓纤维化和真性红细胞增多症。继发于Rux的机会性感染不断有报道；然而，越来越多的研究开始探讨Rux的机制和潜在的免疫抑制作用。病例介绍我们报告了两例在Rux治疗过程中发生在原发性骨髓纤维化患者中的结核病。1例确诊后立即接受Rux治疗，4个月后发现气管、支气管结核和支气管食管瘘。停用Rux后，开始抗结核治疗(ATT)。第二例患者在接受干扰素治疗7.5年后，由于进行性脾肿大而引发RUX，并在2个月后被诊断为播散性结核。他也接受了ATT考试。由于全身症状和脾肿大的高负担，他的Rux得以维持。这些患者的骨髓纤维化和结核病都得到了很好的控制。结论这是首例Rux相关的胆道结核合并支气管食管瘘的病例报告。通过对文献的回顾，我们为骨髓增殖性肿瘤的内在疾病和Rux诱导的免疫松弛共同导致结核病的发现提供了支持证据。我们强调了在Rux治疗前筛查潜伏的结核病感染和及时进行化学预防的重要性。一旦在治疗过程中诊断出结核病，建议停止Rux，并应迅速开始活动的ATT。
Background The selective Janus-activated kinase inhibitor ruxolitinib (rux) is now widely used to treat myelofibrosis and polycythemia vera due to its remarkable effect of reducing splenomegaly and improving constitutional symptoms. With opportunistic infections secondary to rux constantly reported; however, an increasing number of studies have begun to investigate the mechanism and underlying immunosuppressive effect of rux. Case Presentation We report two cases of tuberculosis (TB) in primary myelofibrosis patients during rux therapy. The first patient received rux soon after diagnosis, and tracheobronchial TB (TBTB) and bronchoesophageal fistula were found after 4 months. After discontinuation of rux, antituberculosis therapy (ATT) was introduced. The second patient initiated rux due to progressive splenomegaly after 7.5 years of interferon therapy and was diagnosed with disseminated TB after 2 months. He received ATT as well. His rux was maintained due to the high burden of systematic symptoms and splenomegaly. Both myelofibrosis and TB were well controlled in these patients. Conclusion This is the first case report that describes rux-related TBTB accompanied by a bronchoesophageal fistula. Through a review of the literature, we provide supporting evidence to the finding that intrinsic disorders of myeloproliferative neoplasms and rux-induced immunologic deregulation together lead to TB. We highlight the importance of screening for latent TB infection and timely chemoprophylaxis before rux therapy. Once TB is diagnosed during treatment, rux is recommended to be stopped and active ATT should begin quickly.