关键词: Hemophagocytic lymphohistiocytosis (HLH) case report immune checkpoint inhibitors (ICIs) pembrolizumab predisposing factors Hemophagocytic lymphohistiocytosis (HLH) case report immune checkpoint inhibitors (ICIs) pembrolizumab predisposing factors

来  源:   DOI:10.21037/tcr-22-154   PDF(Pubmed)

Abstract:
UNASSIGNED: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening state of immune hyperactivation. It has the highest mortality rate among all hematological immune-related adverse events (irAEs) of immune checkpoint inhibitors (ICIs) when treating various cancers. However, the predisposing factors of HLH have rarely been mentioned in previous research.
UNASSIGNED: Herein, we report 2 cases of HLH following treatment with pembrolizumab. A patient was diagnosed with thymic carcinoma (TC) and possible Sjögren\'s syndrome (SS), while another was diagnosed with non-small cell lung cancer (NSCLC) and Epstein-Barr virus (EBV) infection, and both were positive for antinuclear antibodies. Both cases experienced transient immune-related fever on day 7 after pembrolizumab administration and splenomegaly on day 10. Then recurrent high-grade fever appeared, and liver function impairment, highly elevated ferritin, and hypertriglyceridemia were tested. After the diagnosis of HLH, both patients were treated with dexamethasone and etoposide without relapse in our follow-up.
UNASSIGNED: Considering the widespread use of ICIs and the high mortality rate of HLH, the immune-related fever, splenomegaly, and other signs of hyperinflammation after the infusion of ICIs, are worthy of attention to the presence of HLH. Preexisting autoimmune diseases (ADs) or positive antibodies, concomitant infection, and the setting of thymic epithelial tumors (TET) may be predisposing factors for HLH. And increased caution is needed before the initiation of ICIs for patients with 2 or more predisposing factors.
摘要:
未经证实:噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见且危及生命的免疫过度激活状态。在治疗各种癌症时,它在免疫检查点抑制剂(ICIs)的所有血液免疫相关不良事件(irAE)中死亡率最高。然而,HLH的诱发因素在以往的研究中很少被提及。
未经批准:此处,我们报告了2例Pembrolizumab治疗后的HLH。一名患者被诊断为胸腺癌(TC)和可能的干燥综合征(SS),而另一个被诊断患有非小细胞肺癌(NSCLC)和EB病毒(EBV)感染,两者的抗核抗体均呈阳性。这两个病例在给予派姆单抗后第7天出现短暂的免疫相关发热,在第10天出现脾肿大。然后出现反复高烧,和肝功能损害,铁蛋白高度升高,和高甘油三酯血症进行了测试。HLH诊断后,在我们的随访中,两名患者均接受地塞米松和依托泊苷治疗,无复发.
未经评估:考虑到ICIs的广泛使用和HLH的高死亡率,免疫相关的发烧,脾肿大,以及输注ICIs后的其他炎症过度症状,HLH的存在值得关注。预先存在的自身免疫性疾病(AD)或抗体阳性,伴随感染,胸腺上皮肿瘤(TET)的形成可能是HLH的诱发因素。对于具有2个或更多个诱发因素的患者,在开始ICIs之前需要更加谨慎。
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