■自身免疫性疾病不是癌症患者免疫检查点抑制剂(ICI)治疗的禁忌症。然而,在接受ICIs的患者中经常观察到免疫相关不良事件(irAE),包括皮炎,甲状腺炎,结肠炎,和肺炎。血小板减少性紫癜,发育不全,在ICI期间很少观察到噬血细胞性淋巴组织细胞增生症(HLH)。
■我们报告了一例男性患者,其预先存在未经治疗的HLAB27和强直性脊柱炎伴胃癌和肝转移。这位79岁的男性接受了抗HER2曲妥珠单抗和抗PD-1纳武单抗治疗。第七个治疗周期后17天,他在急诊科出现急性发烧,混乱,和低血压。实验室结果显示全血细胞减少,以及铁蛋白和甘油三酯的升高。未检测到感染。虽然在骨髓活检中没有看到,临床表现,没有感染,H评分为263,表明HLH。患者接受地塞米松治疗四天,并以逐渐减少的类固醇剂量出院。在两个月的随访中,临床表现正常,血液检查几乎恢复正常.8个月时,未观察到肝转移。
■在预先存在自身免疫性疾病的患者中,免疫疗法导致了HLH的发展,由糖皮质激素控制。骨髓活检中没有吞噬作用的特征并不排除诊断,因为HLH可以发生在脾脏或肝脏中。糖皮质激素治疗不能阻止ICIs的抗癌作用,肝转移在HLH后8个月消失。此病例值得进一步研究自身免疫与ICI反应之间的相互作用,以及ICI诱导的IRAE。
结论:第七周期后曲妥珠单抗(抗HER2)和纳武单抗(抗PD-1)的噬血细胞淋巴组织细胞增生症(HLH)被糖皮质激素控制。耐受性的破坏是由于在患有预先存在的自身免疫性疾病(HLAB27阳性强直性脊柱炎)的患者中免疫疗法诱导的HLH。HLH后8个月肝转移完全消失。
UNASSIGNED: Autoimmune diseases are not contraindications for immune checkpoint inhibitors (ICI) therapy in patients with cancer. However, immune-related adverse events (irAEs) are frequently observed in patients receiving ICIs including dermatitis, thyroiditis, colitis, and pneumonitis. Thrombocytopenic purpura, aplasia, and haemophagocytic lymphohistiocytosis (HLH) are rarely observed during ICIs.
UNASSIGNED: We report the case of a male patient with pre-existing untreated HLA B27 and ankylosing spondylitis with gastric cancer and liver metastases. The 79-year-old man was treated with anti-HER2 trastuzumab and anti-PD-1 nivolumab. Seventeen days after the seventh cycle of treatment, he presented at the emergency department with acute fever, confusion, and hypotension. Laboratory results showed pancytopenia, and elevation of ferritin and triglyceride. No infections were detected. Although not seen in a bone marrow biopsy, clinical presentation, and absence of infection, together with an H-score of 263, indicated HLH. The patient was treated with dexamethasone for four days and discharged on a tapering dose of steroids. At the two-month follow-up, clinical presentation was normal and blood test almost normalised. At 8 months, no liver metastases were observed.
UNASSIGNED: In a patient with a pre-existing autoimmune condition, immunotherapy led to the development of HLH, which was controlled by glucocorticoid. Absence of the feature of haemophagocytosis in the bone marrow biopsy did not exclude the diagnosis, as HLH can occur in the spleen or in the liver. Glucocorticoid therapy did not prevent the anti-cancer effect of ICIs, and liver metastases disappeared 8 months post-HLH. This case warrants further research on the interplay between autoimmunity and ICI response, as well as ICI-induced irAEs.
CONCLUSIONS: Haemophagocytic lymphohistiocytosis (HLH) post seventh cycle of trastuzumab (anti-HER2) and nivolumab (anti-PD-1) was controlled with glucocorticoid.Breach of tolerance was due to immunotherapy-induced HLH in a patient with pre-existing autoimmune condition (HLA B27- positive ankylosing spondylitis).There was a complete disappearance of liver metastases 8 months post-HLH.