■噬血细胞性淋巴组织细胞增生症(HLH)是造血干细胞移植(HSCT)后的罕见并发症。目前,对于移植后HLH的治疗缺乏共识建议.该病例报告强调了成功使用鲁索替尼作为HSCT后HLH的挽救疗法。目的是为这种罕见而复杂的并发症的最佳管理提供有价值的见解。
■我们提供了一个11岁男性患者的案例研究,该患者被诊断为严重的再生障碍性贫血,接受了单倍体相合的HSCT。移植后第86天,病人出现反复发热,肝肿大,高甘油三酯血症,严重的全血细胞减少症,炎症因子和铁蛋白水平升高。在骨髓中观察到吞噬作用,随后的DNA下一代测序确定了腺病毒C型感染,导致腺病毒相关HLH的诊断。在尝试用西多福韦治疗失败后,地塞米松,免疫球蛋白,血浆置换,和依托泊苷,ruxolitinib给药.值得注意的是,患者的临床症状迅速改善,用鲁索替尼治疗,他的测试结果逐渐恢复正常。到第180天,腺病毒病毒载量变得无法检测到。随着持续缓解,ruxolitinib在移植后第137天停药,15个月的随访检查显示无复发。
■我们介绍了一例腺病毒相关的继发性HLH(sHLH)在HSCT后,用鲁索替尼有效治疗。我们的病例强调了鲁索替尼作为病毒感染和sHLH患者的治疗选择的潜力。尽管如此,这种创新治疗的安全性和有效性应在即将进行的大规模临床试验中进行评估.
UNASSIGNED: Hemophagocytic lymphohistiocytosis (HLH) is a rare complication following hematopoietic stem cell transplantation (HSCT). Currently, there is a lack of consensus recommendations for the treatment of post-transplant HLH. This case report emphasizes the successful utilization of ruxolitinib as a salvage therapy for HLH post-HSCT. The aim is to provide valuable insights into the optimal management of this rare and complex complication.
UNASSIGNED: We present a case study of an 11-year-old male patient diagnosed with severe aplastic anemia who received a haploidentical HSCT. On the 86th day post-transplantation, the patient developed recurrent fever, hepatomegaly, hypertriglyceridemia, severe pancytopenia, and elevated levels of inflammatory factors and ferritin. Hemophagocytosis was observed in the bone marrow, and subsequent DNA next-generation sequencing identified adenovirus type C infection, leading to a diagnosis of adenovirus-associated HLH. After unsuccessful treatment attempts with cidofovir, dexamethasone, immunoglobulin, plasmapheresis, and etoposide, ruxolitinib was administered. Remarkably, the patient\'s clinical symptoms rapidly improved, and his test results gradually normalized with ruxolitinib therapy. The adenovirus viral load became undetectable by the 180th day. With continuous remission, ruxolitinib was discontinued on the 137th day post-transplantation, and a 15-month follow-up examination showed no relapse.
UNASSIGNED: We present a case of adenovirus-related secondary HLH (sHLH) post-HSCT, which was effectively treated with ruxolitinib. Our case highlights the potential of ruxolitinib as a therapeutic option for patients with viral infections and sHLH. Nonetheless, the safety and efficacy of this innovative treatment should be evaluated in forthcoming large-scale clinical trials.