title: Central nervous system involvement in hemophagocytic lymphohistiocytosis: a single-center experience.
abstract: BACKGROUND
  Hemophagocytic lymphohistiocytosis is a rare multisystem disorder characterized by proliferation and diffuse infiltration multiple organs with histiocytes, including the central nervous system.
  
  
  METHODS
  Thirty children diagnosed with hemophagocytic lymphohistiocytosis between 1997 and 2010 were reviewed and analyzed. Central nervous system disease involvement was defined as the presence of neurological symptoms and signs or elevated values of cerebrospinal fluid cells and/or proteins.
  
  
  RESULTS
  Among the 30 patients, 17 (56%) had central nervous system involvement. Fourteen patients (46%) presented with neurological symptoms including seizures, irritability, bulging fontanelle, cranial nerve palsy, or disturbance of consciousness, whereas the remaining three patients developed central nervous system symptoms during the course of the disease. Seventeen patients (56%) had cerebrospinal fluid abnormalities. Neuroradiological studies were performed in nine patients. The most common findings were edema, atrophy, subcortical necrosis, and high signal intensity on T2-weighted magnetic resonance imaging. All patients were treated according to the Hemophagocytic Lymphohistiocytosis-94 and Hemophagocytic Lymphohistiocytosis-2004 protocols. Patients with central nervous system involvement had greater mortality. In prediction of the outcome, the cutoff value for cerebrospinal fluid protein was 470 mg/L. The most common neurological sequela was psychomotor delay.
  
  
  CONCLUSIONS
  Central nervous system involvement in hemophagocytic lymphohistiocytosis is common and is associated with poor outcome.
authors: Department of Pediatric Hematology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Neurology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Radiology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Immunology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia.
authors: Ankica
authors: A
authors: Jovanovic
authors: Department of Pediatric Hematology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Neurology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Radiology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Immunology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia.
authors: Milos
authors: M
authors: Kuzmanovic
authors: Department of Pediatric Hematology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Radiology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Immunology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia.
authors: Ruzica
authors: R
authors: Kravljanac
authors: Department of Pediatric Hematology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Neurology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Radiology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Immunology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia.
authors: Dragan
authors: D
authors: Micic
authors: Department of Neurology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Radiology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Immunology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia.
authors: Milena
authors: M
authors: Jovic
authors: Department of Pediatric Hematology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Neurology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Immunology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia.
authors: Slobodan
authors: S
authors: Gazikalovic
authors: Department of Pediatric Hematology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Neurology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Radiology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia. Electronic address: pasics@ikomline.net.
authors: Srdjan
authors: S
authors: Pasic
mesh_terms: D000293:Adolescent; D001921:Brain / Q000473:pathology* / Q000503:physiopathology*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000473:pathology* / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D012372:ROC Curve; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed
keywords: central nervous system; children; hemophagocytic lymphohistiocytosis; outcome