BACKGROUND Hepatosplenic T-cell lymphoma (HSTCL) is a rare, poorly treatable malignancy associated with therapy for IBD. Current knowledge of HSTCL risk in IBD comes from an era of step-up therapy, before earlier use of biologics or combination therapy was advocated to achieve deep mucosal healing. HSTCL risk among newer biologic classes has also not been evaluated. AIMS To systematically characterise the association of HSTCL with biologic therapy for IBD. METHODS We conducted a literature search and query of the Food and Drug Administration Adverse Event Reporting System to summarise HSTCL cases among IBD patients with prior biologic exposure. Demographics and immunosuppression exposure were extracted. Patients were stratified by current regimen (combination therapy, biologic monotherapy or no biologic), and biologic class (anti-TNF, anti-integrin, anti-interleukin 12/23). RESULTS Sixty-two cases of HSTCL were identified from 2486 abstracts and 181 FDA Adverse Events Reporting System reports. The median age of affected patients was 28 years (range 12-81), and 83.6% were male, 84.7% had Crohn's disease. Five of 62 patients had no reported azathioprine/mercaptopurine exposure. Three patients within the cohort developed HSTCL after exposure to natalizumab, vedolizumab or ustekinumab; all three also had anti-TNF and azathioprine/mercaptopurine exposure. Forty-three of 49 (87.8%) patients with known outcomes died with a median survival of 5 months. CONCLUSIONS Consistent with existing data, almost all identified HSTCL cases among IBD patients on biologic therapy had azathioprine/mercaptopurine exposure, and all cases on patients exposed to biologics had anti-TNF exposure. These data suggest initiating a patient-centred discussion before starting anti-TNF therapy or other biologics.

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系统评价：肝脾T细胞淋巴瘤用于炎症性肠病的生物治疗，包括来自美国食品药物管理局不良事件报告系统的数据。

背景技术肝脾性T细胞淋巴瘤（HSTCL）是一种罕见的，难治性的IBD治疗性恶性肿瘤。IBD中HSTCL风险的最新知识来自逐步治疗的时代，在此之前提倡早期使用生物制剂或联合治疗以实现深层粘膜愈合。尚未对新型生物学类别中的HSTCL风险进行评估。目的系统地表征HSTCL与IBD生物疗法的关联。方法我们对美国食品药品管理局不良事件报告系统进行了文献检索和查询，以总结先前有生物接触的IBD患者中的HSTCL病例。提取人口统计学和免疫抑制暴露。根据目前的治疗方案（联合治疗，生物单一疗法或无生物疗法）和生物类别（抗TNF，抗整合素，抗白介素12/23）。结果从2486份摘要和181份FDA不良事件报告系统报告中鉴定出62例HSTCL病例。受影响患者的中位年龄为28岁（范围为12-81岁），其中男性为83.6％，克罗恩病为84.7％。62名患者中有5名没有硫唑嘌呤/巯基嘌呤暴露的报道。该队列中的三名患者在暴露于那他珠单抗，维多珠单抗或乌斯他单抗后发生了HSTCL；这三个人也都有抗TNF和硫唑嘌呤/巯基嘌呤的暴露。已知结局的49例患者中有43例死亡，中位生存期为5个月。结论与现有数据一致，在接受生物疗法的IBD患者中，几乎所有已鉴定的HSTCL病例均接受硫唑嘌呤/巯基嘌呤暴露，而接受生物制剂的所有患者均具有抗TNF暴露。