Division of Hematology and HSCT, Department of Oncology, King Abdul Aziz Medical City, Riyadh, Saudi Arabia.
Division of Hematology and HSCT, Department of Oncology, King Abdul Aziz Medical City, Riyadh, Saudi Arabia; King Abdullah International Medical Research Center, Riyadh, Saudi Arabia; King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.
Hematol Oncol Stem Cell Ther. 2021 Sep;14(3):252-256. doi: 10.1016/j.hemonc.2020.02.003. Epub 2020 Mar 16.
Primary myelofibrosis (PMF) is a subtype of BCR-ABL1 negative myeloproliferative neoplasm. Its characteristic features include clonal myeloproliferation, dysregulation of kinase signaling pathway, abnormal release of cytokines leading to fibrosis in the bone marrow, osteosclerosis, and extramedullary hematopoiesis. Approximately 20% of deaths occur because of disease progression, but death may also result occur because of cardiovascular complications or as a consequence of either infection or bleeding. The only and curative option for PMF is allogeneic hematopoietic stem cell transplant (allo-HSCT); however, the Janus kinase (JAK) 1/2 inhibitor ruxolitinib is highly effective in reducing constitutional symptoms and spleen volume, and has been found to improve survival. Ruxolitinib decreases the activity of type I T-helper cells, leading to decreased release of cytokines including tumor necrosis factor-α, interleukin-1 (IL-1), IL-6, interferon-γ, and production of IL-12, which can be a risk factor for opportunistic infections. In this report, we describe three cases of tuberculosis reactivation shortly after initiation of ruxolitinib therapy followed by a literature review.
原发性骨髓纤维化(PMF)是一种 BCR-ABL1 阴性骨髓增殖性肿瘤。其特征性表现包括克隆性髓系增殖、激酶信号通路失调、细胞因子异常释放导致骨髓纤维化、骨质硬化和髓外造血。约 20%的死亡是由于疾病进展,但也可能因心血管并发症或感染或出血而导致死亡。PMF 的唯一和根治性选择是异基因造血干细胞移植(allo-HSCT);然而,Janus 激酶(JAK)1/2 抑制剂芦可替尼在减轻全身症状和脾脏体积方面非常有效,并已被发现可改善生存。芦可替尼降低了 I 型辅助性 T 细胞的活性,导致包括肿瘤坏死因子-α、白细胞介素-1(IL-1)、IL-6、干扰素-γ和白细胞介素-12 在内的细胞因子释放减少,这可能是机会性感染的一个危险因素。在本报告中,我们描述了三例芦可替尼治疗后不久结核病再激活的病例,并进行了文献复习。