Yabe Mariko, Medeiros L Jeffrey, Daneshbod Yahya, Davanlou Masoud, Bueso-Ramos Carlos E, Moran Elisa J, Young Ken H, Miranda Roberto N
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA; Hematopathology Service, Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Ann Diagn Pathol. 2017 Feb;26:16-22. doi: 10.1016/j.anndiagpath.2016.10.005. Epub 2016 Oct 18.
Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that can arise in patients with underlying immune disorders. Others have suggested that tumor necrosis factor (TNF)-α inhibitor therapy for immune disorders increases the risk of HSTCL. To assess for a potential relationship between HSTCL and the use of TNF-α inhibitors, we searched for patients with HSTCL and underlying immune disorders at our institution. We identified 7 patients with a median age of 38 years. Five patients had Crohn disease, 1 ulcerative colitis, and 1 rheumatoid arthritis. In 6 patients, medication history for the immune disorder was available: 6 patients received 6-mercaptopurine or azathioprine, and 2 patients received steroids; no patients received TNF-α inhibitors. In all 7 patients, the histologic, immunophenotypic, and cytogenetic findings were similar to cases of HSTCL that arise in immunocompetent patients. We reviewed the literature and identified 60 patients with immune disorders who subsequently developed HSTCL. These patients were treated with immunosuppressive drugs in 89%, TNF-α inhibitors in 56%, and both therapies in 54%, and 1 (2%) patient was treated with TNF-α inhibitors only. Our cohort and literature review indicates that TNF-α inhibitor therapy is not essential for the development of HSTCL in patients with immunodysregulatory disorders, and implies that immunosuppressive drugs or other factors (eg, genetic predisposition, chronic antigenic stimulation) may be more critical in the pathogenesis in this context. Although these data are observational, they have implications for the use of TNF-α inhibitors in patients with inflammatory bowel disease and other immunodysregulatory disorders.
肝脾T细胞淋巴瘤（HSTCL）是一种罕见的侵袭性结外T细胞淋巴瘤，可发生于有潜在免疫紊乱的患者。其他人曾提出，用于治疗免疫紊乱的肿瘤坏死因子（TNF）-α抑制剂疗法会增加HSTCL的风险。为了评估HSTCL与TNF-α抑制剂使用之间的潜在关系，我们在本机构中查找患有HSTCL及潜在免疫紊乱的患者。我们确定了7例患者，中位年龄为38岁。5例患有克罗恩病，1例患有溃疡性结肠炎，1例患有类风湿关节炎。在6例患者中，可获取其免疫紊乱的用药史：6例患者接受了6-巯基嘌呤或硫唑嘌呤治疗，2例患者接受了类固醇治疗；无患者接受TNF-α抑制剂治疗。所有7例患者的组织学、免疫表型和细胞遗传学结果均与免疫功能正常患者发生的HSTCL病例相似。我们查阅了文献，确定了60例患有免疫紊乱并随后发生HSTCL的患者。这些患者中，89%接受了免疫抑制药物治疗，56%接受了TNF-α抑制剂治疗，54%接受了两种治疗，仅1例（2%）患者仅接受了TNF-α抑制剂治疗。我们的队列研究和文献综述表明，TNF-α抑制剂疗法对于免疫调节紊乱患者发生HSTCL并非必不可少，这意味着免疫抑制药物或其他因素（如遗传易感性、慢性抗原刺激）在这种情况下的发病机制中可能更为关键。尽管这些数据是观察性的，但它们对炎症性肠病和其他免疫调节紊乱患者使用TNF-α抑制剂具有启示意义。
