Research Article| Volume 357, ISSUE 1-2, P136-142, October 15, 2015

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Neurological involvement and characterization in acquired hemophagocytic lymphohistiocytosis in adulthood


      • Hemophagocytic lymphohistiocytosis is a clinical syndrome characterized by hyperinflammation.
      • HLH affects the nervous system causing a variety of clinical and radiological manifestations.
      • The presence of neurological involvement in HLH may indicate a poor prognosis.
      • HLH can mimic other neuroinflammatory diseases.



      To describe the neurological and neuroradiological features of acquired hemophagocytic lymphohistiocytosis (HLH) in adulthood by reporting a series of cases.


      Ten consecutive patients who were diagnosed with HLH at Medstar Georgetown University Hospital and Walter Reed National Military Medical Center were evaluated for neurological involvement. All underwent clinical neurological evaluation, and when indicated CSF analysis and MR imaging of the brain. Data were gathered and analyzed retrospectively.


      Seven of the ten patients with HLH had neurological involvement. Mean age at onset was 50 (range: 21 to 73). Four patients were males. Prominent clinical features included mild to severe encephalopathy and seizures. Other findings included hemiparesis and spastic tetraparesis. Neuroimaging revealed a wide spectrum of abnormalities including cortical and subcortical edema, gadolinium enhancement, hemorrhage, and diffusion restriction. Basal ganglia involvement was present in four out of seven patients. Three patients died due to multisystem organ failure, and the other patients displayed varying degrees of recovery.


      The neurological features of acquired HLH in adults have not been previously reported. These seven patients demonstrate the spectrum of neurological involvement that can occur. The diagnosis of HLH should be considered in patients who are systemically ill with unexplained fevers and hyperferritinemia who have evidence of inflammation in the CNS.


      HLH (hemophagocytic lymphohistiocytosis), NK-cells (Natural Killer Cells), CSF (cerebrospinal fluid)


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