Department of Neurology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, China.
Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and The Ministry of Education of China, Institute of Neuroscience and the Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, China.
Front Immunol. 2024 Jul 22;15:1383255. doi: 10.3389/fimmu.2024.1383255. eCollection 2024.
This study aims to report three cases of autoimmune encephalitis followed by hemophagocytic lymphohistiocytosis.
Data of relevant patients treated between 2019 and 2022 were retrospectively collected from the Department of Neurology at the Second Affiliated Hospital of Guangzhou Medical University.
The age at onset of the three patients was 37, 63, and 36 years, respectively. All three patients were female and presented with cognitive dysfunction and seizures. Behavioral and psychological symptoms were also observed in two cases. All patients were positive for autoantibodies in both the cerebrospinal fluid and serum, while two showed multiple abnormal brain signals on magnetic resonance imaging. All patients exhibited hypocytosis and elevated soluble CD25 and serum ferritin levels. The final diagnoses in two cases were lymphomas, while the remaining case without tumors suffered from a severe infection. All patients received immunotherapy, and the two with lymphoma received anti-tumor treatment. The patient with infection died, and two patients with tumors improved after chemotherapy.
Autoimmune encephalitis followed by hemophagocytic lymphohistiocytosis is a rare and severe condition. Prompt attention should be paid to the decline in blood cell counts, particularly in patients who show a slight improvement after immunotherapy or have a risk of lymphoma. Screening for potential tumors and infections and early treatment may help these patients.
本研究旨在报告三例自身免疫性脑炎继发噬血细胞性淋巴组织细胞增生症病例。
回顾性收集 2019 年至 2022 年在广州医科大学附属第二医院神经内科治疗的相关患者数据。
3 例患者的发病年龄分别为 37 岁、63 岁和 36 岁，均为女性，表现为认知功能障碍和癫痫发作。2 例患者还出现行为和心理症状。所有患者的脑脊液和血清中均存在自身抗体阳性，2 例患者磁共振成像显示多个异常脑信号。所有患者均出现白细胞减少，可溶性 CD25 和血清铁蛋白水平升高。2 例患者的最终诊断为淋巴瘤，而未发现肿瘤的剩余病例则患有严重感染。所有患者均接受免疫治疗，2 例淋巴瘤患者接受抗肿瘤治疗。感染患者死亡，2 例肿瘤患者经化疗后改善。
自身免疫性脑炎继发噬血细胞性淋巴组织细胞增生症是一种罕见且严重的疾病。应密切关注血细胞计数下降，尤其是在免疫治疗后略有改善或有淋巴瘤风险的患者。筛查潜在肿瘤和感染，并尽早治疗，可能有助于这些患者。
