- •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.
Abbreviations:HLH (hemophagocytic lymphohistiocytosis), NK-cells (Natural Killer Cells), CSF (cerebrospinal fluid)
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to Journal of the Neurological Sciences
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- Familial and acquired hemophagocytic lymphohistiocytosis.Annu. Rev. Med. 2012; 63: 233-246
- Secondary hemophagocytic syndrome in adults: a case series of 18 patients in a single institution and a review of literature.Hematol. Oncol. 2011; 29: 100-106
- The expanding spectrum of hemophagocytic lymphohistiocytosis.Curr. Opin. Allergy Clin. Immunol. 2011; 11: 512-516
- Case 10-2011: a woman with fever, confusion, liver failure, anemia, and thrombocytopenia.N. Engl. J. Med. 2011; 364: 1259-1270
- CNS involvement at the onset of primary hemophagocytic lymphohistiocytosis.Neurology. 2012; 78: 1150-1156
- Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosis.Br. J. Haematol. 2007; 140: 327-335
- Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis.Blood. 1997; 89: 794-800
- HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.Pediatr. Blood Cancer. 2007; 48: 124-131
- Linfohistiocitosis hemofagocitica familiar: hallazgos neuroradiologicos.Radiologia. 2010; 52: 76-80
- A spectrum of neuroradiological findings in children with hemophagocytic lymphohistiocytosis.Pediatr. Radiol. 2007; 37: 1110-1117
- Diffusion-weighted cranial MR imaging findings in a patient with hemophagocytic syndrome.AJNR. 2006; 27: 1312-1314
- Familial hemophagocytic lymphohistiocytosis: clinical and neuroradiological findings and review of the literature.Childs Nerv. Syst. 2010; 26: 121-127
- Callosal and diffuse white matter lesions with restricted water diffusion in hemophagocytic syndrome.Magn. Reson. Med. Sci. 2010; 9: 91-94
- Erdheim–Chester disease of the central nervous system: new manifestations of a rare disease.AJNR Am. J. Neuroradiol. 2011; 32: 2126-2131
- Rosai–Dorfman disease presenting with widespread intracranial and spinal cord involvement.Neurology. 2006; 67: 1551-1555
- Central nervous system involvement in Turkish children with primary hemophagocytic lymphohistiocytosis.J. Child Neurol. 2008; 23: 1293-1299
- Prescribing Information.Novartis Pharmaceuticals Canada Inc., 2012 (www.novartis.ca. Accessed 20 February 2013)
- Pharmacovigilance Risk Assessment Committee meeting minutes.(Accessed 20 February 2013)
- A case of hemophagocytic lymphohistiocytosis syndrome in a patient with Multiple Sclerosis on fingolimod therapy.Neurology. 2014; 82 (Supplement P2.206)
Published online: July 15, 2015
Accepted: July 13, 2015
Received in revised form: June 16, 2015
Received: March 19, 2015
© 2015 Elsevier B.V. Published by Elsevier Inc. All rights reserved.