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Cerebromeningeal haemophagocytic lymphohistiocytosis

J I Henter1, G Elinder

  • 1Department of Paediatrics, Karolinska Institute, St Göran's Children's Hospital, Stockholm, Sweden.

Lancet (London, England)
|January 11, 1992
PubMed

Insights

This study details a progressive encephalopathy in 3 children, an atypical form of hemophagocytic lymphohistiocytosis (HLH). The condition primarily affects the brain and meninges, differing from typical HLH presentations.

Area of Science:

  • Pediatric Neurology
  • Immunology
  • Pathology

Background:

  • Progressive encephalopathy presents with diverse neurological symptoms.
  • Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome.
  • Atypical presentations of HLH can obscure diagnosis.

Observation:

  • Three pediatric cases exhibited progressive encephalopathy with irritability, convulsions, cranial nerve palsies, ataxia, nystagmus, motor deficits, and signs of increased intracranial pressure.
  • Neuropathological examination revealed inflammatory, lymphocytic infiltrates and elevated intracranial pressure.
  • Clinical features were dominated by cerebromeningeal involvement.

Findings:

  • The observed clinical and pathological features represent an atypical variant of hemophagocytic lymphohistiocytosis (HLH).
  • This variant is characterized by primary central nervous system and meningeal inflammation.
  • Cerebromeningeal symptoms preceded the typical systemic manifestations of fever, hepatosplenomegaly, and cytopenias seen in classic HLH.

Implications:

  • Recognizing this atypical HLH presentation is crucial for timely diagnosis and intervention.
  • Early identification of cerebromeningeal involvement can guide treatment strategies.
  • Availability of treatments underscores the importance of distinguishing this condition for improved patient outcomes.

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