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Central nervous system involvement in adults with haemophagocytic lymphohistiocytosis: a single-center study

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder characterized by proliferation and diffuse infiltration multiple organs with histiocytes, including the central nervous system (CNS). Neurological manifestations of HLH have been recognized in different studies with children, but they remain relatively ill-defined in adults with HLH. From March 2008 to October 2014, 289 adult patients with HLH were admitted to our center. Clinical, radiological, and cerebral spinal fluid (CSF) data of the patients with CNS involvement were reviewed, and a retrospective study in our single-center was carried out. CNS involvement was observed in 29 patients (10%) either in their diagnosis process or during disease course. CNS symptoms included disturbance of consciousness, cranial nerve palsies, seizures, headache, limb paralysis, irritability, meningism, and memory loss. CSF analysis was conducted in 17 patients (59%). Among them, 11 patients (65%) were reported as having abnormal CSF. Neuroradiological studies were performed in 25 patients (86%). Among the 13 cases that underwent CT scan, one patient hemorrhaged. Single or multiple hypodense foci were detected in the other 2 patients. Magnetic resonance imaging (MRI) abnormalities were found in 15 patients, including focal lesions in cortical and adjacent subcortical regions with or without variable nodular or ring contrast-enhancement, multiple lesions in white matter, diffuse white matter signal changes, and meningeal enhancement. Basal ganglia, cerebellum, and brainstem lesions were also observed. CNS involvement could also be found in adult patients with HLH, but not as frequent as it was in children. The clinical manifestations could be diversified. By carrying out rigorous CNS examinations, an early diagnosis could be made and it was of the utmost importance for the prevention of further lesions.

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Acknowledgments

The authors would like to thank all the patients for their participation in this study.

This work was supported by the Beijing Science and Technology Plan (grant Z151100004015172), the National Natural Science Fund, China (grant 81401627), and the Medical Development Research Foundation of the Capital, China (grant 2014-4-2025).

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Authors and Affiliations

  1. Department of Neurology, Beijing Friendship Hospital, Capital Medical University, Beijing, China

    Guilan Cai, Xiaojing Liu & Yanfei Han

  2. Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, 100050, China

    Yini Wang & Zhao Wang

Authors
  1. Guilan Cai
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  2. Yini Wang
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  3. Xiaojing Liu
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  4. Yanfei Han
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  5. Zhao Wang
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Corresponding author

Correspondence to Zhao Wang.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participant included in the study.

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The authors declare that they have no conflict of interest.

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Cai, G., Wang, Y., Liu, X. et al. Central nervous system involvement in adults with haemophagocytic lymphohistiocytosis: a single-center study. Ann Hematol 96, 1279–1285 (2017). https://doi.org/10.1007/s00277-017-3035-5

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  • DOI: https://doi.org/10.1007/s00277-017-3035-5

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