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CASE REPORT article

Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders: Autoinflammatory Disorders
Volume 15 - 2024 | doi: 10.3389/fimmu.2024.1383255

Autoimmune encephalitis followed by hemophagocytic lymphohistiocytosis: A cases series

Provisionally accepted
Li Huang Li Huang 1,2Jie Tan Jie Tan 3Peihao Lin Peihao Lin 1,2Zixuan Chen Zixuan Chen 3Qihua Huang Qihua Huang 3Haiyan Yao Haiyan Yao 1,2Lihong Jiang Lihong Jiang 1,2Baoyi Long Baoyi Long 4Youming Long Youming Long 1,2*
  • 1 Department of Neurology, Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, China
  • 2 Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, the Second Affiliated Hospital of Guangzhou Medical University., Guangzhou, China
  • 3 Second Clinical School, Guangzhou Medical University, Guangzhou, China
  • 4 Changsha Medical University, Changsha, Hunan Province, China

The final, formatted version of the article will be published soon.

    Objective: To report three cases of autoimmune encephalitis followed by hemophagocytic lymphohistiocytosis. Methods: 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. Results: The age at onset of the three collected patients were 37, 63, and 36 years. All three patients were female and presented with cognitive dysfunction and seizures. Behavioral and psychological symptoms were also observed in 2 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. Conclusion: 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 slight improvement after immunotherapy, or have a risk of lymphoma. Screening for potential tumors, infections, and early treatment may help these patients.

    Keywords: autoimmune encephalitis, hemophagocytic lymphohistiocytosis, Autoantibody, Lymphoma, Paraneoplastic neurologic syndromes

    Received: 07 Feb 2024; Accepted: 28 Jun 2024.

    Copyright: © 2024 Huang, Tan, Lin, Chen, Huang, Yao, Jiang, Long and Long. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Youming Long, Department of Neurology, Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, China

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